Ujiie Hideyuki

Faculty of Medicine Specialized Medicine Sensory Organ MedicineProfessor
Research Institute for Electronic Science Research Center of Mathematics for Social CreativityProfessor
Hokkaido University HospitalProfessor
Last Updated :2025/06/07

■Researcher basic information

Researchmap personal page

Research Keyword

  • 粘膜類天疱瘡
  • 加齢
  • 制御性T細胞
  • 自己免疫疾患
  • 疾患モデル動物
  • ヒト化マウス
  • 水疱性類天疱瘡
  • 17型コラーゲン
  • サイトカイン
  • ノックアウトマウス
  • Th1/Th2
  • 補体活性化
  • 抗体サブクラス
  • IgG1/IgG4

Research Field

  • Life sciences, Dermatology

Educational Organization

■Career

Career

  • Jan. 2021 - Present
    Hokkaido University, 大学院医学研究院, 教授
  • Sep. 2016 - Dec. 2020
    Hokkaido University, 大学病院, 講師
  • Sep. 2014 - Aug. 2016
    Hokkaido University, 大学病院, 助教
  • Sep. 2012 - Aug. 2014
    NIH, NIAID, Laboratory of Immunology, Visiting fellow
  • Apr. 2010 - Aug. 2012
    Hokkaido University, 大学病院, 助教

Educational Background

  • Apr. 2006 - Mar. 2010, Hokkaido University, 大学院医学研究科 病態制御学専攻, Japan
  • Apr. 1996 - Mar. 2002, Hokkaido University, School of Medicine, 医学科

■Research activity information

Papers

  • Psoriasiform graft-versus-host disease with distal sweat duct involvement as the diagnostic histopathological feature.
    Shinichi Nakazato, Shota Takashima, Toshihiro Matsukawa, Takashi Anan, Hideyuki Ujiie, Yoshihiro Matsuno
    The Journal of dermatology, 19 Dec. 2024, [International Magazine]
    English
  • Spontaneous regression of Merkel cell carcinoma: A case report with pathological and genetic analyses.
    S Hikichi, T Yanagi, S Kitamura, H Nishihara, K Miyamoto, S Mai, K Tokuchi, T Maeda, K Imafuku, H Ujiie
    The Journal of dermatology, 06 Dec. 2024, [International Magazine]
    English
  • Multiple Acantholytic Acanthomas in Junctional Epidermolysis Bullosa.
    Sota Itamoto, Ken Natsuga, Takashi Seo, Shota Takashima, Hideyuki Ujiie
    Acta dermato-venereologica, 104, adv42258, 19 Nov. 2024, [International Magazine]
    English, Scientific journal
  • Spatial confinement induces reciprocating migration of epidermal keratinocytes and forms triphasic epithelia
    Takuma Nohara, Junichi Kumamoto, Yosuke Mai, Mayuna Shimano, Sora Kato, Hiroyuki Kitahata, Hideki Nakamura, Shota Takashima, Mika Watanabe, Masaharu Nagayama, Tsukasa Oikawa, Hideyuki Ujiie, Ken Natsuga
    Cold Spring Harbor Laboratory, 13 Nov. 2024
    Epithelial cells undergo epithelial–mesenchymal transition (EMT) during migration and regain their epithelial phenotype in the post-migration phase (mesenchymal–epithelial transition; MET). We established an experimental system that reproduced three-dimensional triphasic epithelia, i.e., the original epithelium, its EMT, and MET. Keratinocytes (KCs), skin epithelial cells, placed on a microporous membrane migrated through 3.0-um or larger micropores. The 3.0-um-pored membrane induced an epithelial structure with three states: stratified KCs above the membrane, KCs showing EMT within the micropores, and a new stratified epithelium under the membrane. The membrane with larger micropores failed to maintain the triphasic epithelia. Live imaging revealed that KCs moved in a reciprocating manner, with actin-rich filopodia-like KC structures extending into and out of the 3.0-um micropores, while the cells migrated unidirectionally into larger micropores. Piezo1 and keratin 6 were identified as negative modulators of KC entry to and exit from the 3.0-um micropores. These results demonstrate that non-cancerous epithelial cells migrate through confined spaces in a reciprocating manner, which might help form triphasic epithelia, recapitulating wound healing processes.
  • A case of rapidly progressive hair loss due to azathioprine, and the prevalence of NUDT15 variants among Japanese patients with autoimmune blistering diseases: A single-center retrospective observational study.
    Sho Katayama, Kentaro Izumi, Inkin Ujiie, Hideyuki Ujiie
    The Journal of dermatology, 26 Oct. 2024, [International Magazine]
    English, Scientific journal, Autoimmune blistering diseases (AIBDs), classified into pemphigus and pemphigoid, consist of relatively rare skin disorders caused by autoantibodies that target desmosomal and hemidesmosomal proteins, respectively. Although systemic corticosteroids are used as a first-line treatment for AIBDs, azathioprine is frequently co-administered as a steroid-sparing agent. Azathioprine is metabolized into thioguanine nucleotides (TGNs) which are its major active metabolites. The enzyme nudix hydrolase 15 (NUDT15) plays a key role in regulating TGNs. Serious side effects of azathioprine, including leukopenia and alopecia, are known to be particularly problematic in individuals with NUDT15 variants. The single-nucleotide polymorphism c.415C >T (p.Arg139Cys) is one of the most frequent NUDT15 variants associated with severe thiopurine toxicity. Recently, we treated a case of pemphigus vulgaris in a patient with NUDT15 variants in which the patient developed rapidly progressive diffuse hair loss and myelosuppression while receiving azathioprine. Previous reports on NUDT15 polymorphisms mainly focused on patients with inflammatory bowel disease or hematological malignancies, and the prevalence of NUDT15 polymorphisms remains unknown in AIBDs. This highlights the urgent need for research on NUDT15 polymorphisms in AIBDs to achieve a better understanding of the genetic factors influencing adverse reactions to azathioprine. To clarify the prevalence of NUDT15 variants in Japanese patients with AIBDs, we retrospectively reviewed the medical records of 78 patients with AIBDs (26 with bullous pemphigoid, 26 with pemphigus vulgaris, 17 with pemphigus foliaceus, and nine with other AIBDs) who had come to Hokkaido University Hospital between 2018 and 2023. The frequencies of NUDT15 variants of Arg/Arg, Arg/Cys, and Cys/Cys in these patients were approximately 72%, 23%, and 5%, respectively. Our findings indicate a prevalence of NUDT15 variants in AIBD patients that is similar to the prevalences of previous studies on patients with other diseases. These results emphasize the importance of screening for NUDT15 variants prior to initiating azathioprine treatment in Japanese patients with AIBDs.
  • Clinical characteristics and outcomes of dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid patients: A retrospective study.
    Inkin Ujiie, Sho Katayama, Yosuke Mai, Shoko Mai, Norihiro Yoshimoto, Ken Muramatsu, Hiroaki Iwata, Kentaro Izumi, Hideyuki Ujiie
    Journal of the American Academy of Dermatology, 21 Oct. 2024, [International Magazine]
    English, Scientific journal
  • Association between nail psoriasis and obesity: A cross-sectional study at a single institution.
    Sota Itamoto, Hajime Miyazawa, Ken Natsuga, Misako Yamaga, Hiroaki Iwata, Mika Watanabe, Hideyuki Ujiie
    The Journal of dermatology, 30 Sep. 2024, [International Magazine]
    English
  • Type XVII collagen-specific CD4+ T cells induce bullous pemphigoid by producing IL-5.
    Norihiro Yoshimoto, Ken Muramastsu, Takamasa Ito, Miao Zheng, Kentaro Izumi, Ken Natsuga, Hiroaki Iwata, Yoshinori Hasegawa, Hideyuki Ujiie
    The Journal of investigative dermatology, 19 Sep. 2024, [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease caused by anti-type XVII collagen (COL17) antibodies. BP has some immunological features such as eosinophilic infiltration and the deposition of IgE autoantibodies in the skin; however, the mechanism behind such features remains largely unclear. We focused on the autoantigen-specific CD4+ T cells, which are considered to regulate immune response. We established COL17-specific CD4+ T cell lines in vitro. Wild-type mice were immunized with synthesized peptides that include a pathogenic epitope of COL17, and lymphocytes were subjected to a limiting dilution assay. We established 5 T cell lines and examined the pathogenicity by transferring them with COL17-primed B cells into Rag-2-/-/COL17-humanized mice that express human COL17 but not mouse COL17 in the skin. Notably, 3 lines induced BP-like skin changes associated with subepidermal separation and eosinophilic infiltration histologically, and the production of anti-COL17 antibodies. The other 2 lines did not induce such phenotypes. RNA-sequencing analysis revealed that Th2 cytokines, particularly IL-5, were highly expressed in the pathogenic T cell lines. Anti-IL-5 antibody administration significantly reduced the skin changes and attenuated the production of autoantibodies. Thus, the production of IL-5 is critical for COL17-specific CD4+ T cells to induce BP phenotypes in vivo.
  • Patterning in stratified epithelia depends on cell-cell adhesion.
    Yosuke Mai, Yasuaki Kobayashi, Hiroyuki Kitahata, Takashi Seo, Takuma Nohara, Sota Itamoto, Shoko Mai, Junichi Kumamoto, Masaharu Nagayama, Wataru Nishie, Hideyuki Ujiie, Ken Natsuga
    Life science alliance, 7, 9, Sep. 2024, [International Magazine]
    English, Scientific journal, Epithelia consist of proliferating and differentiating cells that often display patterned arrangements. However, the mechanism regulating these spatial arrangements remains unclear. Here, we show that cell-cell adhesion dictates multicellular patterning in stratified epithelia. When cultured keratinocytes, a type of epithelial cell in the skin, are subjected to starvation, they spontaneously develop a pattern characterized by areas of high and low cell density. Pharmacological and knockout experiments show that adherens junctions are essential for patterning, whereas the mathematical model that only considers local cell-cell adhesion as a source of attractive interactions can form regions with high/low cell density. This phenomenon, called cell-cell adhesion-induced patterning (CAIP), influences cell differentiation and proliferation through Yes-associated protein modulation. Starvation, which induces CAIP, enhances the stratification of the epithelia. These findings highlight the intrinsic self-organizing property of epithelial cells.
  • Blaschkoid granulomatous pigmented purpuric dermatosis in childhood.
    George Miura, Mika Watanabe, Hideyuki Kosumi, Takuma Nohara, Ken Natsuga, Hideyuki Ujiie
    The Journal of dermatology, 17 Jul. 2024, [International Magazine]
    English
  • Establishment of a trastuzumab-resistant extramammary Paget disease model: loss of PTEN as a potential mechanism.
    Che-Yuan Hsu, Teruki Yanagi, Takuya Maeda, Hiroshi Nishihara, Takeru Funakoshi, Kodai Miyamoto, Ririko Iwamoto, Kenzo Takahashi, Hideyuki Ujiie
    British journal of cancer, 10 Jul. 2024, [International Magazine]
    English, Scientific journal, BACKGROUND/OBJECTIVES: Extramammary Paget disease (EMPD) is a rare, cutaneous intraepithelial adenocarcinoma typically treated with wide local excision. Unfortunately, a number of patients with metastases show poor responses to chemotherapy. While some studies have explored trastuzumab's effectiveness against EMPD positive for human epidermal growth factor receptor 2 (HER2), trastuzumab resistance (TR) may emerge after anti-HER2 therapy. METHODS/SUBJECTS: In this study, we established TR EMPD patient-derived xenografts (PDX) that replicated the histological and HER2 expression traits of naive EMPD tumours. RESULTS: Cancer gene analyses revealed a loss of the PTEN gene in TR tumours, which was further confirmed by immunohistochemical staining and immunoblotting to test for protein expression levels. Reduced PTEN levels correlated with increased protein kinase B (Akt) phosphorylation and p27 downregulation, suggesting a potential mechanism for trastuzumab resistance in EMPD cells. In the trastuzumab-sensitive EMPD-PDX mouse model, PTEN inhibitors partially restored trastuzumab-mediated tumour regression. The TR EMPD-PDX responded favourably to targeted therapy (lapatinib, abemaciclib, palbociclib) and chemotherapy (eribulin, docetaxel, trastuzumab deruxtecan). CONCLUSIONS: This study demonstrates an innovative TR EMPD-PDX model and introduces promising antineoplastic effects with various treatments for TR EMPD tumours.
  • Study Design of a Phase 2/3 Randomized Controlled Trial of Dupilumab in Adults with Bullous Pemphigoid: LIBERTY-BP ADEPT.
    Dédée F Murrell, Pascal Joly, Victoria P Werth, Hideyuki Ujiie, Margitta Worm, Aaron R Mangold, Elena Avetisova, Jennifer Maloney, Elizabeth Laws, Eric Mortensen, Ariane Dubost-Brama, Arsalan Shabbir
    Advances in therapy, 41, 7, 2991, 3002, Jul. 2024, [International Magazine]
    English, Scientific journal, BACKGROUND: Bullous pemphigoid (BP) is a rare, autoimmune, blistering skin disease associated with high disease burden, profoundly decreased quality of life and increased morbidity. Emerging evidence supports an important role for type 2 inflammation in disease pathogenesis. Current management relies on topical and/or systemic corticosteroids, non-selective immunosuppressants and antibiotics with anti-inflammatory properties, which are all limited by side effects and toxicities. Therefore, targeted, efficacious and safe therapies are needed. Dupilumab blocks the shared receptor component for interleukin (IL)-4 and IL-13, key and central drivers of type 2 inflammation. Several reports of patients successfully treated with dupilumab have been published; however, dupilumab has not been formally assessed in a double-blind, placebo-controlled trial. OBJECTIVES: We report the design of LIBERTY-BP ADEPT, a multicenter, randomized, double-blind, placebo-controlled clinical trial to evaluate the efficacy and safety of dupilumab in adults with BP. METHODS: LIBERTY-BP ADEPT comprises a 35-day screening, 52-week treatment and 12-week follow-up period. Approximately 98 adults aged 18-90 years with moderate-to-severe BP are being enrolled at 51 sites on 4 continents and randomized 1:1 to subcutaneous dupilumab or placebo every 2 weeks. All participants will receive concomitant oral corticosteroids (OCS). PLANNED OUTCOMES: The primary endpoint is the proportion of patients achieving complete remission off steroid therapy at week 36. Key secondary endpoints include total cumulative OCS dose to week 36, percent change and proportion of patients with ≥ 4-point reduction in the weekly average of daily Peak Pruritus Numerical Rating Scale from baseline to week 36 and percent change in Bullous Pemphigoid Area Index score from baseline to week 36. CONCLUSION: The trial results will provide evidence on whether the efficacy and safety of dupilumab support its use as a potential novel treatment approach for BP and will provide new insights into the role of type 2 inflammation in BP pathogenesis. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT04206553.
  • Correction to: Study Design of a Phase 2/3 Randomized Controlled Trial of Dupilumab in Adults with Bullous Pemphigoid: LIBERTY-BP ADEPT.
    Dédée F Murrell, Pascal Joly, Victoria P Werth, Hideyuki Ujiie, Margitta Worm, Aaron R Mangold, Elena Avetisova, Jennifer Maloney, Elizabeth Laws, Eric Mortensen, Ariane Dubost-Brama, Arsalan Shabbir
    Advances in therapy, 41, 7, 3014, 3015, Jul. 2024, [International Magazine]
    English
  • Atypical manifestations of hemangiomas in epidermolysis bullosa.
    S Itamoto, K Natsuga, S Takashima, H Ujiie
    The Journal of dermatology, 14 Jun. 2024, [International Magazine]
    English
  • 【環状の紅斑と環状紅斑】環状血管炎 review of the literature
    長田 悠里, 宮澤 元, 得地 景子, 眞井 翔子, 泉 健太郎, 氏家 英之
    皮膚病診療, 46, 6, 540, 543, (株)協和企画, Jun. 2024
    Japanese
  • 水疱性類天疱瘡における好塩基球活性化と末梢血好塩基球数の低下はBullous Pemphigoid Disease Area Index(BPDAI)と相関する               
    川村 拓也, 泉 健太郎, 眞井 洋輔, 眞井 翔子, 片山 奨, 葭本 倫大, 氏家 韻欣, 氏家 英之, 渡辺 陽久
    日本皮膚科学会雑誌, 134, 7, 1912, 1912, (公社)日本皮膚科学会, Jun. 2024
    Japanese
  • 水疱性類天疱瘡における好塩基球活性化と末梢血好塩基球数の低下はBullous Pemphigoid Disease Area Index(BPDAI)と相関する               
    川村 拓也, 泉 健太郎, 眞井 洋輔, 眞井 翔子, 片山 奨, 葭本 倫大, 氏家 韻欣, 氏家 英之, 渡辺 陽久
    日本皮膚科学会雑誌, 134, 7, 1922, 1922, (公社)日本皮膚科学会, Jun. 2024
    Japanese
  • SpO2低下により診断し得たジアフェニルスルホンによるメトヘモグロビン血症の2例               
    黒澤 卓, 宮澤 元, 渡邉 美佳, 長田 悠里, 眞井 翔子, 得地 景子, 今福 恵輔, 氏家 英之, 木村 彩萌, 伊藤 圭
    日本皮膚科学会雑誌, 134, 6, 1657, 1657, (公社)日本皮膚科学会, May 2024
    Japanese
  • 抗BP180抗体を有する疱疹状天疱瘡の1例               
    濱 芙美, 眞井 洋輔, 眞井 翔子, 小住 英之, 片山 奨, 氏家 韻欣, 泉 健太郎, 柳 輝希, 氏家 英之, 小玉 和郎
    日本皮膚科学会雑誌, 134, 6, 1658, 1658, (公社)日本皮膚科学会, May 2024
    Japanese
  • Native collagen XVII complex ELISA: An approach for diagnosis and monitoring of anti‐integrin β4 mucous membrane pemphigoid
    S. Mai, K. Izumi, S. Itamoto, S. Kurosawa, Y. Nagata, S. Hikichi, H. Miyazawa, K. Tokuchi, K. Imafuku, T. Yanagi, M. Tsutsui, H. Ujiie
    Journal of the European Academy of Dermatology and Venereology, May 2024
    Scientific journal
  • Pseudolymphoma on the face involving the conjunctiva.
    Akihiro Orita, Shota Takashima, Norihiro Yoshimoto, Atsushi Narahira, Yosuke Mai, Ken Arita, Satoru Kase, Wataru Nishie, Hideyuki Ujiie
    Clinical and experimental dermatology, 49, 5, 521, 523, 23 Apr. 2024, [International Magazine]
    English, Scientific journal
  • Autologous sweat-induced anaphylaxis and basophil activation test.
    Kentaro Izumi, Wataru Nishie, Yasuyuki Yamaguchi, Yu Hirata, Teruki Yanagi, Hideyuki Ujiie
    Journal of the European Academy of Dermatology and Venereology : JEADV, 22 Apr. 2024, [International Magazine]
    English
  • Morphea-like subcutaneous panniculitis-like T-cell lymphoma.
    Keiko Tokuchi, Teruki Yanagi, Emi Inamura, Takuya Kawamura, Takashi Seo, Yasuyuki Fujita, Masao Nakagawa, Ikumi Kasahara, Yoshihiro Matsuno, Hideyuki Ujiie
    JAAD case reports, 46, 27, 29, Apr. 2024, [International Magazine]
    English
  • Clinical-histopathological features and cancer gene analysis of cutaneous epithelioid angiosarcoma: A report of 4 cases.
    Keiko Tokuchi, Teruki Yanagi, Suguru Kurosawa, Shinya Kitamura, Takuya Maeda, Che Yuan Hsu, Kodai Miyamoto, Hiroshi Nishihara, Hideyuki Ujiie
    JAAD case reports, 46, 45, 48, Apr. 2024, [International Magazine]
    English
  • Successful treatment of multicentric Castleman's disease associated with dystrophic epidermolysis bullosa using anti-interleukin-6 receptor antibody.
    Satsuki Naruse, Shota Takashima, Yasuyuki Fujita, Hiroshi Kataoka, Nobuaki Kawamura, Ken Natsuga, Hideyuki Ujiie
    The Journal of dermatology, 14 Mar. 2024, [International Magazine]
    English
  • Spatial transcriptomics of pemphigus vulgaris and bullous pemphigoid: Insights into pathogenesis and therapy on bullous formation.
    Joong Heon Suh, Seo-Young Choi, Yun Jung Huh, Jung Ho Lee, So-Jung Choi, Chung-Gyu Park, Hyun Je Kim, Hideyuki Ujiie, Seon-Pil Jin, Jeong Eun Kim
    Journal of the European Academy of Dermatology and Venereology : JEADV, 03 Mar. 2024, [International Magazine]
    English
  • Association between weight loss and death in patients with malignant melanoma: A retrospective study of 28 cases.
    Arisa Tanaka, Hajime Miyazawa, Teruki Yanagi, Takuya Maeda, Shinya Kitamura, Hideyuki Ujiie
    The Journal of dermatology, 51, 3, 463, 466, Mar. 2024, [International Magazine]
    English, Scientific journal, Malignant melanoma (MM) is often associated with a poor prognosis due to metastasis and cancer death. The monitoring of prognostic factors is of vital importance, and among these factors, elevated lactate dehydrogenase (LDH) should be closely observed during the disease course. Important factors for predicting the survival of MM patients include tumor thickness, ulceration, the number of lymph node metastases, metastatic lesions, and the sites of metastasis. Weight loss is not generally included in the prognostic factors of MM, but it is monitored in other cancers, such as lung cancer and gastrointestinal cancer. The objective of this study was to investigate the association between weight loss and MM prognosis. Using data from MM patients who had been treated at our institution, we assessed the prognoses of two groups: weight loss of at least 5% body weight or weight loss not exceeding 5% body weight within a 12-month period. As a result, a higher mortality rate was found for the former group. Furthermore, the loss of at least 5% of body weight within a month was found to almost always adversely affect the patient's prognosis. The present study indicates that there may be an association between MM prognosis and weight loss of at least 5% within a year, and body weight could potentially serve as an informative factor for MM survival.
  • A familial case of extramammary Paget disease: Analysis of whole-exome sequencing
    Takuya Maeda, Teruki Yanagi, Shinya Kitamura, Hiroshi Nishihara, Yusuke Ono, Yusuke Mizukami, Shinya Tanaka, Hideyuki Ujiie
    EJC Skin Cancer, Mar. 2024, [Peer-reviewed]
  • Multiple courses of steroid pulse therapy are required in treating acquired idiopathic generalized anhidrosis patients with a large anhidrotic area: A retrospective study of 28 cases.
    Keisuke Imafuku, Teruki Yanagi, Norihiro Yoshimoto, Hajime Miyazawa, Hiroaki Iwata, Hideyuki Ujiie
    The Australasian journal of dermatology, 65, 1, 55, 58, Feb. 2024, [International Magazine]
    English, Scientific journal, Patients with acquired idiopathic generalized anhidrosis (AIGA) demonstrate a sudden loss of sweating function without neurological or endocrine abnormalities. The main treatment is steroid pulse therapy. However, the number of courses required for improvement has been unclear. This study aims to clarify the factors associated with AIGA disease severity and with AIGA patients' responses to steroid pulse therapy. We retrospectively analysed the clinical information of 28 patients with AIGA in our department from the last 10 years. Univariate analysis revealed that patients with a large anhidrotic area need multiple courses of steroid pulse therapy.
  • 略全身の乾癬様紅色局面               
    中里 信一, 高島 翔太, 松川 敏大, 阿南 隆, 氏家 英之, 松野 吉宏
    日本皮膚病理組織学会抄録集, 40回, 52, 52, 日本皮膚病理組織学会, Jan. 2024
    Japanese
  • Dermoscopic Features of Heterotopic Salivary Gland Tissue in the Neck: Report of a Case.
    Shinichiro Inoue, Takuya Maeda, Yuri Nagata, Teruki Yanagi, Hideyuki Ujiie
    Case reports in dermatology, 16, 1, 17, 20, 2024, [International Magazine]
    English, INTRODUCTION: Heterotopic salivary gland tissue (HSGT) is diagnosed when salivary gland tissue is found in areas other than the major or minor salivary glands. No dermoscopic findings of HSGT have been reported. CASE PRESENTATION: This case report gives the dermoscopic findings of a 45-year-old woman with HSGT who presented with a longstanding pale red macule on her neck, characterized by repeated clear fluid discharge. Dermoscopic examination showed a red dot with a pale pink halo and telangiectasia throughout the lesion. An excisional biopsy confirmed the diagnosis of HSGT, revealing the presence of mucous and serous salivary glandular structures within subcutaneous fat tissue. It has been reported that HSGT is associated with periductal lymphocytic infiltration, which was also observed in this case. CONCLUSION: We think that the "red dot with a pale pink halo" is a unique dermoscopic finding of HSGT, which is rarely observed in branchial anomalies.
  • Eribulin for patients with metastatic extramammary Paget disease: Study protocol for a single-arm phase II trial.
    Takuya Maeda, Teruki Yanagi, Keiko Tokuchi, Takeru Funakoshi, Nao Horie, Toshiyuki Isoe, Yoichi M Ito, Norihiro Sato, Hideyuki Ujiie
    Experimental dermatology, 33, 1, e14993, Jan. 2024, [International Magazine]
    English, Scientific journal, Extramammary Paget disease (EMPD) is a rare cutaneous malignancy that predominantly affects the anogenital areas of the elderly. Although the efficacy of docetaxel and other cytotoxic agents for advanced EMPD has been reported in small retrospective case studies, no treatment has been proven effective in prospective clinical trials. We established the world's first in vivo EMPD experimental model (a patient-derived xenograft model). In our treatment experiment, xenograft tumours showed a remarkable response to eribulin. This study evaluates the efficacy of eribulin for patients with advanced EMPD. In October 2022, we started a single-arm phase II trial to evaluate the efficacy of eribulin as a treatment for adult patients with unresectable EMPD with measurable lesions. Enrolment in this clinical trial is open to patients with any prior treatment for EMPD. The primary endpoint is overall response rate; the secondary endpoints include disease control rate, overall survival, progression-free survival and adverse events. The study protocol was approved by the Ethics Committee of Hokkaido University and the other collaborating institutions. If the primary endpoint is met, it is our hope that eribulin will be regarded as a standard medication for patients with advanced EMPD.
  • Transcriptomic response of peripheral blood mononuclear cells to dupilumab in a 65-year-old patient with bullous pemphigoid.
    Meng-Ling Li, Yi-Kai Hong, Yu-Chen Lin, Ken Natsuga, Hideyuki Ujiie, Kentaro Izumi, Hiroaki Iwata, Chao-Kai Hsu
    Clinical and experimental dermatology, 49, 1, 73, 74, 19 Dec. 2023, [International Magazine]
    English, Scientific journal
  • Eribulin inhibits growth of cutaneous squamous cell carcinoma cell lines and a novel patient-derived xenograft
    Che Yuan Hsu, Teruki Yanagi, Takuya Maeda, Hiroshi Nishihara, Kodai Miyamoto, Shinya Kitamura, Keiko Tokuchi, Hideyuki Ujiie
    Scientific Reports, 13, 1, 8650, 8650, Dec. 2023, [International Magazine]
    English, Scientific journal
  • Association of Genetic Variants of HLA-DQA1 with Bullous Pemphigoid Induced by Dipeptidyl Peptidase-4 Inhibitors.
    Takeshi Ozeki, Ken Muramatsu, Norihiro Yoshimoto, Inkin Ujiie, Kentaro Izumi, Hiroaki Iwata, Taisei Mushiroda, Hideyuki Ujiie
    The Journal of investigative dermatology, 143, 11, 2219, 2225, Nov. 2023, [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP) is the most common autoimmune blistering disorder. Several factors, including an antidiabetic (dipeptidyl peptidase-4 inhibitor [DPP-4i]), have been reported to trigger BP. To identify the genetic variants associated with BP, GWAS and HLA fine-mapping analyses were conducted. The 21 cases of noninflammatory BP induced by DPP-4i (i.e., DPP-4i-induced noninflammatory BP) and 737 controls (first cohort) and the 8 cases and 164 controls (second cohort) were included in the GWAS. Combining GWAS satisfied the genome-wide significant association of HLA-DQA1 (chromosome 6, rs3129763 [T/C]) with the risk of DPP-4i-induced noninflammatory BP (allele T carrier of 72.4% [21 of 29] in cases vs. 15.3% [138 of 901] in controls; dominant model, OR = 14, P = 1.8 × 10-9). HLA fine mapping revealed that HLA-DQA1∗05 with serine at position 75 of HLA-DQα1 (Ser75) had the most significant association with the combined cohort of DPP-4i-induced noninflammatory BP (79.3% [23 of 29] cases vs. 16.1% [145 of 901] controls; dominant model, OR = 21, P = 2.0 × 10-10). HLA-DQα1 Ser75 polymorphism was located inside the functional pocket of HLA-DQ molecules, suggesting the impact of HLA-DQα1 Ser75 on DPP-4i-induced noninflammatory BP.
  • Hand-foot syndrome histopathologically presenting eccrine squamous syringometaplasia due to pembrolizumab after lenvatinib treatment.
    Sota Itamoto, Keisuke Imafuku, Hajime Miyazawa, Takashi Anan, Hiroko Matsumiya, Daisuke Endo, Takashi Mitamura, Hideyuki Ujiie
    Journal of cutaneous pathology, 50, 11, 932, 935, Nov. 2023, [International Magazine]
    English, Scientific journal
  • Dermal discoloration due to osmium tetroxide.
    Satsuki Naruse, Shota Takashima, Ken Natsuga, Hideyuki Ujiie
    Clinical toxicology (Philadelphia, Pa.), 61, 11, 1004, 1005, Nov. 2023, [International Magazine]
    English, Scientific journal, INTRODUCTION: Osmium tetroxide is a strong oxidizing agent. After dermal exposure to osmium tetroxide, skin discoloration and red papules can occur. We describe a patient with skin discoloration due to osmium tetroxide. CASE SUMMARY: A 25-year-old postgraduate student unintentionally exposed his hand to osmium tetroxide while working in a laboratory setting. After immediate washing, he sought medical care due to left middle finger discoloration. He reported no discomfort in the affected area. Thorough water rinsing was continued, and corticosteroid ointment was applied. IMAGES: Our patient developed dark brown pigmentation on the ventral side of the left middle finger. The pigmentation disappeared one week later. CONCLUSION: Osmium tetroxide may induce dark brown skin discoloration.
  • Development of a nutritionally balanced, melt‐in‐the‐mouth chocolate for patients with epidermolysis bullosa
    Kosei Nakamura, Shota Takashima, Takuma Nohara, Mika Watanabe, Ken Natsuga, Hideyuki Ujiie
    The Journal of Dermatology, 50, 12, 1640, 1643, Wiley, 07 Sep. 2023, [International Magazine]
    English, Scientific journal, Abstract

    Epidermolysis bullosa (EB) is a group of inherited blistering disorders that primarily affect the skin and mucous membranes of the digestive tract, which can lead to poor nutritional status. Dietary supplements and nutritional support methods, such as nasogastric tubes and gastrostomy, have been employed to improve the nutritional status of patients with EB; however, few foods are suitable for enjoyable eating with family and friends. Here, we introduce a nutritionally balanced, melt‐in‐the‐mouth chocolate called andew, which was specifically designed for patients with EB. The andew chocolate is nutritionally superior and melts more easily than traditional chocolates, thus it is suitable for patients with EB, who are prone to oral erosions. Patients responded more favorably to the taste and texture of andew than to those of other dietary supplements. Not only does andew provide nutritional benefits, but it also promotes enjoyable eating with family members and friends, which could positively impact patients' mental health.
  • A case of bullous pemphigoid developing under treatment with benralizumab for bronchial asthma.
    Arisa Tanaka, Yu Fujimura, Satoshi Fuke, Kentaro Izumi, Hideyuki Ujiie
    The Journal of dermatology, 50, 9, 1199, 1202, Sep. 2023, [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP) is an autoimmune disease characterized by itchy erythema and tense blisters on the whole body. Recent reports have unveiled the pathogenic roles of eosinophils in BP (e.g., dermal-epidermal separation, generation of pruritus). Thus, eosinophils are considered a therapeutic target. Benralizumab is an anti-IL-5 receptor alpha (IL-5Rα) monoclonal antibody (mAb) that is widely used to treat severe eosinophilic asthma. By affecting IL-5Rα, benralizumab depletes eosinophils and basophils due to apoptosis through antibody-dependent cell-mediated cytotoxicity. The efficacies of benralizumab and other biologics, including bertilimumab (anti-eotaxin-1 mAb) and mepolizumab (anti-IL-5 mAb), were evaluated in several clinical trials. Also, reslizumab, an anti-IL-5 mAb, was reported as a successful treatment option in a case of BP. We present a case of severe asthma treated with benralizumab at 8-week intervals for 3 years before BP developed. Histologically, subepidermal blisters without eosinophilic infiltration were observed. Methylprednisolone pulse therapy followed by 40 mg/day (1 mg/kg/day) of oral prednisolone (PSL) was initiated, but the skin lesions worsened. Additional intravenous immunoglobulin and oral azathioprine enabled the oral PSL to be tapered. The benralizumab was discontinued after the onset of BP because the asthma did not worsen. To the best of our knowledge, there have been no reports of BP developing during anti-eosinophil therapy. BP may occur paradoxically via various pathways during treatment with drugs that are typically effective against BP.
  • 水疱性類天疱瘡とBullous Rheumatoid Neutrophilic Dermatosisとの鑑別を要したリウマトイド血管炎
    田中 有沙, 柳 輝希, 今福 恵輔, 渥美 達也, 氏家 英之
    皮膚科の臨床, 65, 10, 1531, 1534, 金原出版(株), Sep. 2023
    Japanese
  • Detection of a natural antibody targeting the shed ectodomain of BP180 in mice.
    Yosuke Mai, Kentaro Izumi, Shoko Mai, Wataru Nishie, Hideyuki Ujiie
    Journal of dermatological science, 112, 1, 15, 22, 01 Aug. 2023, [International Magazine]
    English, Scientific journal, BACKGROUND: Pemphigoid diseases are characterized by subepidermal blister formation accompanied by autoantibodies targeting skin component molecules, such as BP180. It is suggested that an epitope-phenotype correlation exists among autoantibodies recognizing BP180. However, it is unclear which regions of BP180 are likely targets for autoantibodies. OBJECTIVE: To elucidate the portions of BP180 where antibodies tend to react under the breakdown of immune tolerance. METHODS: We immunized mice with full-length mouse BP180 (mBP180) to produce anti-mBP180 antibodies. Using the immunized mice, hybridoma cells were established to produce anti-mBP180 antibodies. We analyzed the characteristics of the anti-mBP180 antibodies that were produced in terms of epitopes, immunoglobulin subclasses, and somatic hypermutations. RESULTS: Hybridoma cells derived from immunized mice with full-length mBP180 produced antibodies targeting the intracellular domain (IC) and the shed ectodomain (EC) of mBP180. Using the domain-deleted mBP180 recombinant protein, we revealed that monoclonal anti-mBP180 EC antibodies react to neoepitopes on the 13th collagenous region of cleaved mBP180, which corresponds to the epitopes of linear IgA bullous dermatosis antibodies in human BP180. Furthermore, the subclasses of these antibodies could be distinguished by epitope: The subclass of the anti-mBP180 IC monoclonal antibodies was IgG, whereas that of the anti-mBP180 EC antibodies was IgM. Of note, a clone of these IgM mBP180 EC antibodies was a germline antibody without somatic hypermutation, which is also known as a natural antibody. CONCLUSION: These data suggest that mice potentially have natural antibodies targeting the neoepitopes of cleaved mBP180 EC.
  • Prognostic Analysis of Patients With Extramammary Paget Disease Treated With Conservative Excision
    Takashi Seo, Shinya Kitamura, Teruki Yanagi, Takuya Maeda, Hideyuki Ujiie
    Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.], 49, 8, 743, 746, 01 Aug. 2023, [International Magazine]
    English, Scientific journal
  • Skin biopsies for diagnosing neuronal intranuclear inclusion disease: A retrospective study of 12 cases.
    Sota Itamoto, Teruki Yanagi, Ichiro Yabe, Yoshihiro Matsuno, Hideyuki Ujiie
    The Journal of dermatology, 50, 7, 931, 934, Jul. 2023, [International Magazine]
    English, Scientific journal, Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative condition. Skin biopsies taken from the lower leg were reported to be a standard diagnostic procedure for NIID; however, no studies have addressed the optimal skin biopsy locations. We retrospectively analyzed 12 cases in which skin biopsies were performed for diagnosing NIID. We collected clinical information including age, sex, skin biopsy site, the presence of nuclear inclusion bodies, the results of p62 immunostaining, the final diagnosis from the department of neurology, and the presence of abnormal GGC repeats in the NOTCH2NLC gene. Four of the 12 cases had a final diagnosis of NIID. One of the four cases was biopsied from the lower leg, whereas the other three cases were biopsied from the abdomen or thigh. Biopsy specimens of the four definite NIID cases revealed the average rates of nuclear inclusion body-positive cells in adipocytes, sweat gland cells, and fibroblasts to be 13.2%, 10.3%, and 6.3%, respectively. GGC repeat abnormalities in the NOTCH2NLC gene were observed in two of the four cases. The present study indicates that sites with ample subcutaneous fat tissue could be promising for diagnostic skin biopsies for NIID.
  • Lessons from clinical trials on triple combination of immune checkpoint inhibitors and BRAF/MEK inhibitors in BRAF-mutant melanoma.
    Takuya Maeda, Teruki Yanagi, Hideyuki Ujiie
    Annals of translational medicine, 11, 9, 326, 326, 30 Jun. 2023, [International Magazine]
    English
  • 姑息的切除を行った乳房外パジェット病の予後に関する検討               
    瀬尾 拓志, 北村 真也, 柳 輝希, 前田 拓哉, 氏家 英之
    加齢皮膚医学セミナー, 18, 1, 42, 42, 加齢皮膚医学研究会, Jun. 2023
    Japanese
  • 体重減少は悪性黒色腫の予後不良因子になりうる               
    田中 有沙, 宮澤 元, 前田 拓哉, 北村 真也, 柳 輝希, 氏家 英之
    西日本皮膚科, 85, 3, 233, 234, 日本皮膚科学会-西部支部, Jun. 2023
    Japanese
  • 姑息的切除を行った乳房外パジェット病の予後に関する検討               
    瀬尾 拓志, 北村 真也, 柳 輝希, 前田 拓哉, 氏家 英之
    加齢皮膚医学セミナー, 18, 1, 42, 42, 加齢皮膚医学研究会, Jun. 2023
    Japanese
  • Pseudotumor of the skin due to Mycobacterium genavense.
    Suguru Kurosawa, Keisuke Imafuku, Sho Nakakubo, Sumio Iwasaki, Takanori Teshima, Hideki Goto, Hideyuki Ujiie
    International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases, 134, 88, 90, 15 May 2023, [International Magazine]
    English, Mycobacterium genavense is a rare type of non-tuberculous mycobacterium (NTM) that has been reported to cause disseminated infections in immunocompromised patients. Because M. genavense is slow-growing and poorly able to form colonies on Ogawa's medium, genetic and molecular analyses are necessary to identify this pathogen. NTM infections present with various cutaneous manifestations. Of these, rare cases have been reported to present with mycobacterial pseudotumors. However, there are no reports of M. genavense with cutaneous pseudotumors. In this paper, we report a case of a pseudotumor due to M. genavense infection that was observed only in a cutaneous lesion. The patient was taking 5 mg of prednisolone and was aware of a tumor on the right lower leg. Biopsy samples showed diffuse spindle-shaped histiocytes and various other inflammatory cell infiltrates, and Ziehl-Neelsen staining detected mycobacterium. Since no colonies formed on Ogawa medium, genetic testing was performed, and M. genavense was identified by DNA sequence analysis. There were no other disseminated lesions beyond the skin, including in the lungs and liver. Since the patient was immunosuppressed, in accordance with previous literature, a combination therapy of clarithromycin, ethambutol, and rifampicin for 4 months was recommended. When no growth is observed on Ogawa's medium in cases of infection, it is essential to identify the infectious pathogen by genetic analysis.
  • 家族性乳房外パジェット病における遺伝子変異解析結果               
    前田 拓哉, 柳 輝希, 西原 広史, 小野 裕介, 水上 裕輔, 田中 伸哉, 氏家 英之
    日本皮膚科学会雑誌, 133, 5, 1350, 1350, (公社)日本皮膚科学会, May 2023
    Japanese
  • 基底膜タンパク複合体を利用したELISAで診断し得た粘膜類天疱瘡の1例               
    眞井 翔子, 泉 健太郎, 板本 想太, 黒澤 卓, 長田 悠里, 挽地 史織, 宮澤 元, 得地 景子, 今福 恵輔, 柳 輝希, 筒井 眞人, 氏家 英之
    日本臨床皮膚科医会雑誌, 40, 3, 477, 477, 日本臨床皮膚科医会, May 2023
    Japanese
  • Mycobacterium genavenseによる限局性の皮膚感染症を呈した1例               
    黒澤 卓, 今福 恵輔, 宮澤 元, 中久保 祥, 岩崎 澄央, 後藤 秀樹, 豊嶋 崇徳, 氏家 英之
    日本臨床皮膚科医会雑誌, 40, 3, 470, 470, 日本臨床皮膚科医会, May 2023
    Japanese
  • Native Autoantigen Complex Detects Pemphigoid Autoantibodies.
    Shoko Mai, Kentaro Izumi, Yosuke Mai, Ken Natsuga, Norito Ishii, Daisuke Sawamura, Franziska Schauer, Dimitra Kiritsi, Wataru Nishie, Hideyuki Ujiie
    JID innovations : skin science from molecules to population health, 3, 3, 100193, 100193, May 2023, [International Magazine]
    English, Scientific journal, Pemphigoid diseases are a group of autoimmune disorders characterized by subepidermal blistering in the skin and mucosa. Among them, mucous membrane pemphigoid (MMP) autoantibodies are characterized by targeting multiple molecules in the hemidesmosomes, including collagen XVII, laminin-332, and integrin a6/β4. Traditionally, recombinant proteins of the autoantigens have been employed to identify circulating autoantibodies by immune assays. However, developing an efficient detection system for MMP autoantibodies has been challenging because the autoantibodies have heterogeneous profiles and the antibody titers are typically low. In this study, we introduce an ELISA that takes advantage of a native autoantigen complex rather than simple recombinant proteins. We generated HaCaT keratinocytes with a DDDDK-tag knocked in at the COL17A1 locus by CRISPR/Cas9-mediated gene editing. Immunoprecipitation using the DDDDK-tag isolated a native complex that contained full-length and processed collagen XVII and integrin α6/β4. Then, we used the complex proteins to prepare an ELISA system and enrolled 55 MMP cases to validate its diagnostic performance. The sensitivity and specificity of the ELISA for detecting MMP autoantibodies were 70.9% and 86.7%, respectively, far superior to those of conventional assays. In autoimmune diseases such as MMP, in which autoantibodies target various molecules, isolating the antigen-protein complexes can help establish a diagnostic system.
  • Two cases of Hallopeau-type pemphigus vegetans with anti-desmoglein 1 and anti-desmocollin 3 antibodies without mucosal involvement.
    Takuya Kawamura, Ken Muramatsu, Akihiro Orita, Yosuke Mai, Tatsuro Sugai, Naoya Haga, Yu Fujimura, Toshinari Miyauchi, Kentaro Izumi, Hiroshi Koga, Norito Ishii, Hideyuki Ujiie
    Journal of the European Academy of Dermatology and Venereology : JEADV, 37, 4, e508-e510, Apr. 2023, [International Magazine]
    English
  • イミキモドクリームが奏効した基底細胞母斑症候群の2例
    小川 弘記, 北村 真也, 田中 有沙, 山賀 三紗子, 平野 瑶子, 瀬尾 拓志, 葭本 倫大, 加藤 直子, 柳 輝希, 氏家 英之
    臨床皮膚科, 77, 4, 347, 352, (株)医学書院, Apr. 2023
    Japanese
  • Systemic Retinoids for Generalized Verrucosis Due to Congenital Immunodeficiency: Case Reports and Review of the Literature.
    Hideyuki Kosumi, Ken Natsuga, Teruki Yanagi, Hideyuki Ujiie
    Genes, 14, 3, 22 Mar. 2023, [International Magazine]
    English, Scientific journal, Generalized verrucosis (GV) is a group of immunodeficiency disorders accompanied by widespread human papillomavirus infection. We revisit two cases of GV due to congenital interleukin-7 deficiency successfully treated with systemic retinoids. We also present a review of the literature on the use of systemic retinoids to treat GV. Our review suggests that systemic retinoids are a safe and effective option for managing recalcitrant wart lesions in cases of GV.
  • Zonula occludens‐1 distribution and barrier functions are affected by epithelial proliferation and turnover rates
    Keisuke Imafuku, Hiroaki Iwata, Ken Natsuga, Makoto Okumura, Yasuaki Kobayashi, Hiroyuki Kitahata, Akiharu Kubo, Masaharu Nagayama, Hideyuki Ujiie
    Cell Proliferation, 56, 9, e13441, Wiley, 14 Mar. 2023, [International Magazine]
    English, Scientific journal, Zonula occludens-1 (ZO-1) is a scaffolding protein of tight junctions, which seal adjacent epithelial cells, that is also expressed in adherens junctions. The distribution pattern of ZO-1 differs among stratified squamous epithelia, including that between skin and oral buccal mucosa. However, the causes for this difference, and the mechanisms underlying ZO-1 spatial regulation, have yet to be elucidated. In this study, we showed that epithelial turnover and proliferation are associated with ZO-1 distribution in squamous epithelia. We tried to verify the regulation of ZO-1 by comparing normal skin and psoriasis, known as inflammatory skin disease with rapid turnover. We as well compared buccal mucosa and oral lichen planus, known as an inflammatory oral disease with a longer turnover interval. The imiquimod (IMQ) mouse model, often used as a psoriasis model, can promote cell proliferation. On the contrary, we peritoneally injected mice mitomycin C, which reduces cell proliferation. We examined whether IMQ and mitomycin C cause changes in the distribution and appearance of ZO-1. Human samples and mouse pharmacological models revealed that slower epithelial turnover/proliferation led to the confinement of ZO-1 to the uppermost part of squamous epithelia. In contrast, ZO-1 was widely distributed under conditions of faster cell turnover/proliferation. Cell culture experiments and mathematical modelling corroborated these ZO-1 distribution patterns. These findings demonstrate that ZO-1 distribution is affected by epithelial cell dynamics.
  • Ashy dermatosis with a Wagyu beef‐like dermoscopic appearance: Report of two cases
    Yuki Munekata, Teruki Yanagi, Mayuna Shimano, Akihiro Orita, Chihiro Shiiya, Shinya Kitamura, Hideyuki Ujiie
    JEADV Clinical Practice, Mar. 2023
    Scientific journal
  • ニボルマブによる術後補助療法を行った末端黒子型悪性黒色腫患者5名の治療効果の検討               
    前田 拓哉, 北村 真也, 柳 輝希, 氏家 英之
    日本皮膚科学会雑誌, 133, 3, 505, 505, (公社)日本皮膚科学会, Mar. 2023
    Japanese
  • 乳房外パジェット病(EMPD)の前臨床モデルに対する抗HER2抗体薬物複合体(抗HER2ADC)の治療実験               
    得地 景子, 柳 輝希, 前田 拓哉, 北村 真也, 氏家 英之
    日本皮膚科学会雑誌, 133, 3, 539, 539, (公社)日本皮膚科学会, Mar. 2023
    Japanese
  • What's new in the pathogeneses and triggering factors of bullous pemphigoid.
    Hideyuki Ujiie
    The Journal of dermatology, 50, 2, 140, 149, Feb. 2023, [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP) is a subepidermal blistering disease induced by autoantibodies to type XVII collagen (COL17, also called BP180) and BP230. Previous studies using patients' samples and animal disease models elucidated the complement-dependent and complement-independent pathways of blister formation, the pathogenic roles of immune cells (T and B cells, macrophages, mast cells, neutrophils, eosinophils), and the pathogenicity of IgE autoantibodies in BP. This review introduces the recent progress on the mechanism behind the epitope-spreading phenomenon in BP, which is considered to be important to understand the chronic and intractable disease course of BP, and the pathogenicity of anti-BP230 autoantibodies, mainly focusing on studies that used active disease models. To clarify the pathogenesis of BP, the mechanism behind the breakdown of immune tolerance to BP antigens should be investigated. Recent studies using various experimental models have revealed important roles for regulatory T cells in the maintenance of self-tolerance to COL17 and BP230 as well as in the suppression of inflammation triggered by the binding of antibodies to COL17. Notably, physical stresses such as trauma, thermal burns, bone fractures, irradiation and ultraviolet exposure, some pathologic conditions such as neurological diseases and hematological malignancies, and the use of dipeptidyl peptidase-IV inhibitors and immune checkpoint inhibitors have been reported as triggering factors for BP. These factors and certain underlying conditions such as genetic background, regulatory T-cell dysfunction or aging might synergistically affect some individuals and eventually induce BP. Further studies on the breakdown of self-tolerance and on the identification of key molecules that are relevant to blister formation and inflammation may expand our understanding of BP's etiology and may lead to the development of novel therapeutic approaches.
  • FAM83Hの発現低下はケラチン分布の変化を介して皮膚有棘細胞癌の遊走と浸潤を促進する               
    得地 景子, 北村 真也, 前田 拓哉, 氏家 英之, 柳 輝希, 渡部 昌, 畠山 鎮次, 加納 里志, 田中 伸哉
    日本皮膚科学会雑誌, 133, 2, 265, 265, (公社)日本皮膚科学会, Feb. 2023
    Japanese
  • Intravenous immunoglobulin in patients with bullous pemphigoid insufficient response to corticosteroids: Nationwide post-marketing surveillance in Japan.
    Hideyuki Ujiie, Makoto Arakawa, Yumi Aoyama
    Journal of dermatological science, 109, 1, 22, 29, Jan. 2023, [International Magazine]
    English, Scientific journal, BACKGROUND: In Japan, intravenous immunoglobulin (IVIG) has been approved for corticosteroid-unresponsive bullous pemphigoid (BP); however, its usage, efficacy, and safety in clinical settings remain unclear. OBJECTIVE: To elucidate IVIG efficacy, we examined the improvement in disease severity based on the Bullous Pemphigoid Disease Area Index (BPDAI). METHODS: In this 3-year (April 2016-March 2019), prospective, post-marketing, observational surveillance study, we enrolled 379 patients (51.3 % men; mean age, 74.5 years) with corticosteroid-unresponsive BP treated with IVIG from 143 institutions in Japan (720 treatment cycles). The percentage of patients who improved by at least one severity stage or whose symptoms completely resolved based on the BPDAI score was evaluated at 15, 30, and 60-90 days. RESULTS: The improvement rates at 15, 30, and 60-90 days after initial treatment in the 328 IVIG-naïve patients were 70.7 %, 83.5 %, and 84.3 %, respectively. The BPDAI score decreased rapidly and significantly by 15 days compared with that observed during pre-treatment. Further improvement was observed at 30 and 60-90 days. The corticosteroid dose and anti-BP180 antibody titers decreased significantly post-treatment (both, p < .001). Approximately 25 % of IVIG-naïve patients underwent multiple treatment cycles. The improvement rate at 30 days after the final dose was 88 %, and the symptoms completely resolved in 44 % of patients. The incidence of adverse drug reactions per cycle was 8.34 %; the most common reaction was transient thrombocytopenia. CONCLUSION: Most patients showed improvement in severity and decrease in corticosteroid dose and anti-BP180 antibody levels post-treatment, indicating that IVIG is useful for corticosteroid-unresponsive BP treatment.
  • Beneficial effects of nintedanib on cardiomyopathy in patients with systemic sclerosis: a pilot study.
    Keita Ninagawa, Masaru Kato, Satonori Tsuneta, Suguru Ishizaka, Hideyuki Ujiie, Ryo Hisada, Michihito Kono, Yuichiro Fujieda, Yoichi M Ito, Tatsuya Atsumi
    Rheumatology (Oxford, England), 62, 7, 2550, 2555, 02 Dec. 2022, [International Magazine]
    English, Scientific journal, OBJECTIVES: Nintedanib is an inhibitor of tyrosine kinases that has been shown to slow the progression of interstitial lung disease (ILD), including ILD associated with systemic sclerosis (SSc). The aim of this study was to explore the effect of nintedanib on cardiomyopathy associated with SSc. METHODS: Twenty consecutively hospitalized patients with SSc-ILD were enrolled and prospectively followed. The rate of change at six months in cardiac magnetic resonance (CMR) parametric mapping, including myocardial extracellular volume, was primarily evaluated. Other endpoints included changes in CMR functional parameters, echocardiographic parameters, modified Rodnan skin score, serum biomarkers, and pulmonary function test. RESULTS: Nintedanib was administered in 10 patients, whereas the other 10 were treated without nintedanib or watched, according to ILD severity and progression. Baseline values of CMR parametric mapping were not different between the two groups. The rate of change at six months in myocardial extracellular volume was largely different, almost divergent between the nintedanib group and the control group (-1.62% vs. +2.00%, p= 0.0001). Among other endpoints, the change in right ventricular ejection fraction was significantly different between the two groups (p= 0.02), with a preferential change in the nintedanib group. CONCLUSION: Our data indicate beneficial signals of nintedanib on cardiomyopathy associated with SSc. The anti-fibrotic effect of nintedanib might not be limited to the lung.
  • Giant basal cell carcinoma of the scrotum: A case report and review of the literature
    Hiroki Ogawa, Shinya Kitamura, Takashi Seo, Norihiro Yoshimoto, Teruki Yanagi, Hideyuki Ujiie
    JEADV Clinical Practice, Dec. 2022
    Scientific journal
  • Carotenoderma due to lycopenemia: A case report and evaluation of lycopene deposition in the skin.
    Arisa Tanaka, Toshinari Miyauchi, Shinya Kitamura, Hiroaki Iwata, Hiroo Hata, Hideyuki Ujiie
    The Journal of dermatology, 49, 12, 1320, 1324, Dec. 2022, [International Magazine]
    English, Scientific journal, Carotenoderma is a yellow-orange coloration of the skin caused by high levels of serum carotenoids, mostly due to the excessive intake of carotenoid-rich foods. The yellowish coloration is typically observed on the palms, soles, and nasolabial folds. Although the physical appearance is prominent, the condition itself is benign and harmless. Diagnosing carotenoderma is not difficult because of its unique manifestations, but its pathophysiology remains unclear. We report a relatively rare case of carotenoderma due to lycopenemia caused by the excessive intake of lycopene-rich vegetables and fruits. Lycopene is a carotenoid component that is distinguished by the high absorption of light around 488 nm. Given these characteristics, we examined a hematoxylin-eosin-stained specimen from the patient and tape-stripped samples by fluorescent microscopy with 488 nm wavelength emission and compared them with normal skin samples. Notably, the patient's samples showed a weaker autofluorescence in the stratum corneum and sweat glands. Furthermore, we measured carotenoid concentrations in the patient's skin noninvasively with Vegecheck® and found a higher score than the average of 24 healthy volunteers. These results support the long-held hypothesis that carotenoids are secreted in sweat and are deposited in the stratum corneum. To the best of our knowledge, no previous reports have measured skin carotenoid levels nor detailed the pathological findings of carotenoderma patients. This case further highlights that the excessive intake of lycopene causes carotenoderma and demonstrates that carotenoid deposition is particularly pronounced in the stratum corneum of the skin.
  • A case of atopic dermatitis-associated dupilumab-resistant alopecia totalis successfully treated with baricitinib.
    Takashi Seo, Kentaro Izumi, Takuya Kawamura, Masumi Tsujiwaki, Hideyuki Ujiie
    Dermatologic therapy, 35, 12, e15953, Dec. 2022, [International Magazine]
    English
  • CDK4/6阻害剤は乳房外Paget病の腫瘍増殖を抑制する               
    北村 真也, 前田 拓哉, 柳 輝希, 氏家 英之
    西日本皮膚科, 84, 6, 576, 576, 日本皮膚科学会-西部支部, Dec. 2022
    Japanese
  • 陰茎リンパ浮腫上に生じたHPV52型陽性Bowen病の1例               
    田中 有沙, 北村 真也, 瀬尾 拓志, 葭本 倫大, 柳 輝希, 氏家 英之
    日本皮膚科学会雑誌, 132, 12, 2696, 2696, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • 壊疽性膿皮症が疑われた壊疽性膿瘡の2例               
    田中 有沙, 柳 輝希, 小川 弘記, 瀬尾 拓志, 小住 英之, 眞井 翔子, 宮澤 元, 今福 恵輔, 夏賀 健, 氏家 英之, 箱崎 茉衣
    日本皮膚科学会雑誌, 132, 12, 2697, 2698, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • 姑息的切除を行った乳房外パジェット病の予後に関する検討               
    瀬尾 拓志, 北村 真也, 柳 輝希, 氏家 英之
    日本皮膚科学会雑誌, 132, 12, 2698, 2698, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • 陰嚢に生じた巨大基底細胞癌の1例               
    小川 弘記, 北村 真也, 平野 瑶子, 田中 有沙, 瀬尾 拓志, 葭本 倫大, 柳 輝希, 氏家 英之, 梅本 浩平
    日本皮膚科学会雑誌, 132, 12, 2698, 2698, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • イミキモドクリームが奏効した基底細胞母斑症候群の2例               
    小川 弘記, 北村 真也, 田中 有沙, 山賀 三紗子, 平野 瑶子, 瀬尾 拓志, 葭本 倫大, 柳 輝希, 氏家 英之, 加藤 直子
    日本皮膚科学会雑誌, 132, 12, 2700, 2700, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • 好塩基球活性化試験がアレルゲンの同定に有用であった自己汗アナフィラキシーの1例               
    泉 健太郎, 柳 輝希, 氏家 英之, 西江 渉, 山口 泰之, 平田 悠
    日本皮膚科学会雑誌, 132, 12, 2702, 2702, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • Cutaneous epithelioid angiosarcomaの4例               
    黒澤 卓, 前田 拓哉, 北村 真也, 柳 輝希, 氏家 英之, 稲村 衣美, 安藤 佐土美, 小林 仁, 安岡 厚
    日本皮膚科学会雑誌, 132, 12, 2704, 2704, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • 神経核内封入体病診断における皮膚生検の検討               
    板本 想太, 柳 輝希, 氏家 英之, 矢部 一郎, 松野 吉宏
    日本皮膚科学会雑誌, 132, 12, 2704, 2704, (公社)日本皮膚科学会, Nov. 2022
    Japanese
  • Adjuvant nivolumab therapy may not improve disease-free survival in resected acral lentiginous melanoma patients: A retrospective case series
    Takuya Maeda, Teruki Yanagi, Kodai Miyamoto, Keiko Tokuchi, Shinya Kitamura, Hideyuki Ujiie
    Dermatologic Therapy, 35, 11, e15817, Nov. 2022, [International Magazine]
    English, Scientific journal
  • Rituximab therapy for intractable pemphigus: A multicenter, open-label, single-arm, prospective study of 20 Japanese patients.
    Jun Yamagami, Yuichi Kurihara, Takeru Funakoshi, Yasuko Saito, Ryo Tanaka, Hayato Takahashi, Hideyuki Ujiie, Hiroaki Iwata, Yoji Hirai, Keiji Iwatsuki, Norito Ishii, Jun Sakurai, Takayuki Abe, Ryo Takemura, Naomi Mashino, Masahiro Abe, Masayuki Amagai
    The Journal of dermatology, 50, 2, 175, 182, 04 Oct. 2022, [International Magazine]
    English, Scientific journal, This was a multicenter clinical trial of rituximab, a chimeric monoclonal IgG antibody directed against CD20, for the treatment of refractory pemphigus vulgaris and pemphigus foliaceus. In total, 20 patients were treated with two doses of rituximab (1000 mg; 2 weeks apart) on days 0 and 14. The primary end point was the proportion of patients who achieved complete or partial remission on day 168 following the first rituximab dose. Of the 20 enrolled patients, 11 (55%) and four (20%) achieved complete and partial remission, respectively; therefore, remission was achieved in a total of 15 patients (75.0% [95% confidence interval, 50.9%-91.3%]). It was demonstrated that the remission rate was greater than the prespecified threshold (5%). In addition, a significant improvement in clinical score (Pemphigus Disease Area Index) and decrease in serum anti-desmoglein antibody level were observed over time. Four serious adverse events (heart failure, pneumonia, radial fracture, and osteonecrosis) were recorded in two patients, of which only pneumonia was considered causally related with rituximab. The level of peripheral blood CD19-positive B lymphocytes was decreased on day 28 after rituximab treatment and remained low throughout the study period until day 168. Our results confirm the efficacy and safety of rituximab therapy for refractory pemphigus in Japanese patients.
  • Acquired perforating dermatosis induced by necitumumab.
    A Kimura, H Kosumi, K Natsuga, T Goda, H Ujiie
    Journal of the European Academy of Dermatology and Venereology : JEADV, 36, 10, e822-e823, Oct. 2022, [International Magazine]
    English
  • Multiple actinic keratosis on the face associated with pirfenidone-induced photosensitivity.
    Yoko Hirano, Shinya Kitamura, Takashi Seo, Norihiro Yoshimoto, Teruki Yanagi, Hideyuki Ujiie
    The Journal of dermatology, 49, 8, e253-e254, Aug. 2022, [International Magazine]
    English
  • Ecthyma gangrenosum caused by Streptococcus pyogenes: Report of a case and review of the literature.
    Arisa Tanaka, Teruki Yanagi, Takashi Seo, Shoko Mai, Hajime Miyazawa, Hideyuki Ujiie
    The Journal of dermatology, 49, 8, e266-e267, Aug. 2022, [International Magazine]
    English
  • Apremilast for lithium-associated psoriasis
    Joohyung Youh, Hajime Miyazawa, Hiroaki Iwata, Ken Natsuga, Hideyuki Ujiie
    Dermatologica Sinica, Jul. 2022, [Peer-reviewed]
    English, Scientific journal
  • HER2-Targeted Antibody–Drug Conjugates Display Potent Antitumor Activities in Preclinical Extramammary Paget’s Disease Models: In Vivo and Immunohistochemical Analyses
    Keiko Tokuchi, Takuya Maeda, Shinya Kitamura, Teruki Yanagi, Hideyuki Ujiie
    Cancers, 14, 14, Jul. 2022, [International Magazine]
    English, Scientific journal
  • Regression of acquired reactive perforating collagenosis after dipeptidyl peptidase-4 inhibitor cessation.
    Takuya Kawamura, Yosuke Mai, Atsushi Narahira, Yoshihiro Matsuno, Wataru Nishie, Hideyuki Ujiie
    The Journal of dermatology, 49, 7, e238-e240, Jul. 2022, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • Human figure‐shaped contact dermatitis due to the illustration on the inner surface of compression sleeves
    Takashi Seo, Toshinari Miyauchi, Tsuyoshi Kawakami, Hideyuki Ujiie
    The Journal of Dermatology, 49, 7, e241-e242, Wiley, Jul. 2022, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • Wnt/β-Catenin Signaling Stabilizes Hemidesmosomes in Keratinocytes.
    Hideyuki Kosumi, Mika Watanabe, Satoru Shinkuma, Takuma Nohara, Yu Fujimura, Tadasuke Tsukiyama, Giacomo Donati, Hiroaki Iwata, Hideki Nakamura, Hideyuki Ujiie, Ken Natsuga
    The Journal of investigative dermatology, 142, 6, 1576, 1586, Jun. 2022, [Peer-reviewed], [Internationally co-authored], [International Magazine]
    English, Scientific journal, Hemidesmosomes (HDs) are adhesion complexes that promote epithelial-stromal attachment in stratified and complex epithelia, including the epidermis. In various biological processes, such as differentiation and migration of epidermal keratinocytes during wound healing or carcinoma invasion, quick assembly and disassembly of HDs are prerequisites. In this study, we show that inhibition of Wnt/β-catenin signaling disturbs HD organization in keratinocytes. Screening with inhibitors identified the depletion of HD components and HD-like structures through Wnt inhibition, but keratinocyte differentiation was not affected. Wnt inhibition significantly diminished plectin and type XVII collagen expression in the basal side of Wnt-inhibited cells and the dermo-epidermal junction of the Wnt-inactive murine basal epidermis. Similar to Wnt inhibition, PLEC-knockout cells or cells with plectin-type XVII collagen binding defects showed type XVII collagen reduction in the basal side of the cells, implying the possible involvement of Wnt/β-catenin signaling in HD assembly. Atypical protein kinase C inhibition ameliorated the phenotypes of Wnt-inhibited cells. These findings show that Wnt/β-catenin signaling regulates the localization of HD components in keratinocytes and that the atypical protein kinase C pathway is involved in Wnt inhibition‒induced HD disarrangement. Our study suggests that the Wnt signaling pathway could be a potential therapeutic target for treating HD-defective diseases, such as epidermolysis bullosa.
  • Case Report: Hereditary Fibrosing Poikiloderma With Tendon Contractures, Myopathy, and Pulmonary Fibrosis (POIKTMP) Presenting With Liver Cirrhosis and Steroid-Responsive Interstitial Pneumonia
    Michiko Takimoto-Sato, Toshinari Miyauchi, Masaru Suzuki, Hideyuki Ujiie, Toshifumi Nomura, Tomoo Ikari, Tomohiko Nakamura, Kei Takahashi, Machiko Matsumoto-Sasaki, Hirokazu Kimura, Hiroki Kimura, Yuichiro Matsui, Takashi Kitagataya, Ren Yamada, Kazuharu Suzuki, Akihisa Nakamura, Masato Nakai, Takuya Sho, Koji Ogawa, Naoya Sakamoto, Naoko Yamaguchi, Noriyuki Otsuka, Utano Tomaru, Satoshi Konno
    Frontiers in Genetics, 13, 870192, 870192, Frontiers Media SA, 05 May 2022, [Peer-reviewed], [International Magazine]
    English, Scientific journal, Background: Hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis (POIKTMP) is an extremely rare disease caused by mutations in FAM111B, and only approximately 30 cases have been reported worldwide. Some patients develop interstitial pneumonia, which may lead to progressive pulmonary fibrosis and poor prognosis. However, no effective treatment for interstitial pneumonia associated with POIKTMP has been reported. Here, we report an autopsy case of POIKTMP, wherein interstitial pneumonia was improved by corticosteroids.

    Case Presentation: A 44-year-old Japanese man was referred to our hospital due to poikiloderma, hypotrichosis, and interstitial pneumonia. He developed progressive poikiloderma and muscle weakness since infancy. He also had tendon contractures, short stature, liver cirrhosis, and interstitial pneumonia. Mutation analysis of FAM111B revealed a novel and de novo heterozygous missense mutation, c.1886T &gt; G (p(Phe629Cys)), through which we were able to diagnose the patient with POIKTMP. 3 years after the POIKTMP diagnosis, interstitial pneumonia had worsened. After 2 weeks of administrating 40 mg/day of prednisolone, his symptoms and lung shadows improved. However, he subsequently developed severe hepatic encephalopathy and eventually died of respiratory failure due to bacterial pneumonia and pulmonary edema. Autopsy revealed an unclassifiable pattern of interstitial pneumonia, as well as the presence of fibrosis and fatty degeneration in several organs, including the liver, kidney, skeletal muscle, heart, pancreas, and thyroid.

    Conclusions: We report a case of POIKTMP in which interstitial pneumonia was improved by corticosteroids, suggesting that corticosteroids could be an option for the treatment of interstitial pneumonia associated with this disease.
  • Case of penile Bowen's disease on postoperative lymphedema associated with human papilloma virus type 52.
    Arisa Tanaka, Shinya Kitamura, Teruki Yanagi, Takashi Seo, Norihiro Yoshimoto, Hideyuki Ujiie
    The Journal of dermatology, 49, 5, e163-e164, May 2022, [International Magazine]
    English
  • Dermoscopic features of aneurysmal dermatofibroma: A case report and review of the literature.
    Yuki Munekata, Shinya Kitamura, Teruki Yanagi, Mayuna Shimano, Hideyuki Ujiie
    The Journal of dermatology, 49, 5, e169-e170, May 2022, [International Magazine]
    English
  • 当科でパルス療法を施行した特発性後天性全身性無汗症患者の解析               
    今福 恵輔, 柳 輝希, 葭本 倫大, 宮澤 元, 岩田 浩明, 氏家 英之
    日本皮膚科学会雑誌, 132, 5, 1307, 1307, (公社)日本皮膚科学会, May 2022
    Japanese
  • BP180と7型コラーゲンを標的抗原とした免疫チェックポイント阻害薬関連表皮下水疱症の1例               
    葭本 倫大, 平野 瑶子, 得地 景子, 泉 健太郎, 松本 隆児, 氏家 英之
    日本皮膚科学会雑誌, 132, 5, 1310, 1310, (公社)日本皮膚科学会, May 2022
    Japanese
  • BP180と7型コラーゲンを標的抗原とした免疫チェックポイント阻害薬関連表皮下水疱症の1例               
    葭本 倫大, 平野 瑶子, 得地 景子, 泉 健太郎, 松本 隆児, 氏家 英之
    日本皮膚科学会雑誌, 132, 5, 1310, 1310, (公社)日本皮膚科学会, May 2022
    Japanese
  • Cas9-guided haplotyping of three truncation variants in autosomal recessive disease.
    Ken Natsuga, Yoshikazu Furuta, Shota Takashima, Takuma Nohara, Hsin-Yu Huang, Satoru Shinkuma, Hideki Nakamura, Yousuke Katsuda, Hideaki Higashi, Chao-Kai Hsu, Satoshi Fukushima, Hideyuki Ujiie
    Human mutation, 43, 7, 877, 881, 21 Apr. 2022, [Peer-reviewed], [Last author], [Internationally co-authored], [International Magazine]
    English, Scientific journal, An autosomal recessive disease is caused by biallelic loss-of-function mutations. However, when more than two disease-causing variants are found in a patient's gene, it is challenging to determine which two of the variants are responsible for the disease phenotype. Here, to decipher the pathogenic variants by precise haplotyping, we applied nanopore Cas9-targeted sequencing (nCATS) to three truncation COL7A1 variants detected in a patient with recessive dystrophic epidermolysis bullosa (EB). The distance between the most 5' and 3' variants was approximately 19 kb at the level of genomic DNA. nCATS successfully demonstrated that the most 5' and 3' variants were located in one allele while the variant in between was located in the other allele. Interestingly, the proband's mother, who was phenotypically intact, was heterozygous for the allele that harbored the two truncation variants, which could otherwise be misinterpreted as those of typical recessive dystrophic EB. Our study highlights the usefulness of nCATS as a tool to determine haplotypes of complicated genetic cases. Haplotyping of multiple variants in a gene can determine which variant should be therapeutically targeted when nucleotide-specific gene therapy is applied.
  • Toxic epidermal necrolysis induced by tirabrutinib.
    Yoko Hirano, Hideyuki Kosumi, Hajime Miyazawa, Teruki Yanagi, Takahiro Hase, Ayame Kimura, Makoto Saito, Takeshi Kondo, Hideyuki Ujiie
    The Journal of dermatology, 49, 4, e135-e136, Apr. 2022, [International Magazine]
    English
  • Detection of revertant mosaicism in epidermolysis bullosa through Cas9-targeted long-read sequencing.
    Ken Natsuga, Yoshikazu Furuta, Shota Takashima, Takuma Nohara, Hideyuki Kosumi, Yosuke Mai, Hideaki Higashi, Hideyuki Ujiie
    Human mutation, 43, 4, 529, 536, Apr. 2022, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal, Revertant mosaicism (RM) is a phenomenon in which inherited mutations are spontaneously corrected in somatic cells. RM occurs in some congenital skin diseases, but genetic validation of RM in clinically revertant skin has been challenging, especially when homologous recombination (HR) is responsible for RM. Here, we introduce nanopore Cas9-targeted sequencing (nCATS) for identifying HR in clinically revertant skin. We took advantage of compound heterozygous COL7A1 mutations in a patient with recessive dystrophic epidermolysis bullosa who showed revertant skin spots. Cas9-mediated enrichment of genomic DNA (gDNA) covering the two mutation sites (>8 kb) in COL7A1 and subsequent MinION sequencing successfully detected intragenic crossover in the epidermis of the clinically revertant skin. This method enables the discernment of haplotypes of up to a few tens of kilobases of gDNA. Moreover, it is devoid of polymerase chain reaction amplification, which can technically induce recombination. We, therefore, propose that nCATS is a powerful tool for understanding complicated gene modifications, including RM.
  • Characteristic Linear and Zigzag Purpuric Lesions in a Patient with Long-term and Repeated Exposure to Bedbug Bites.
    Akihiro Orita, Toshinari Miyauchi, Inkin Ujiie, Hideyuki Ujiie
    Acta dermato-venereologica, 102, adv00661, 08 Mar. 2022, [International Magazine]
    English, Scientific journal
  • Efficacy of a combination of paclitaxel and radiation therapy against cutaneous angiosarcoma: A single-institution retrospective study of 21 cases.
    Takashi Seo, Shinya Kitamura, Teruki Yanagi, Hideyuki Ujiie
    The Journal of dermatology, 49, 3, 383, 386, Mar. 2022, [International Magazine]
    English, Scientific journal, Cutaneous angiosarcoma (CAS) is a rare malignant tumor with a poor prognosis for which neither basic research on molecular pathomechanisms nor clinical prospective studies have progressed. A retrospective study of 28 CAS cases reported that chemoradiotherapy with taxanes was superior to conventional surgery. Since that time, chemoradiotherapy with paclitaxel (PTX + RT) has become a standard treatment. In this paper, we retrospectively investigate 21 cases of CAS that had been treated in the Department of Dermatology at Hokkaido University Hospital. Patients initially treated with PTX + RT followed by maintenance taxane chemotherapy showed better prognosis in overall survival (OS) and progression-free survival (PFS) (median OS, 28 months; median PFS, 12 months) compared to others (median OS, 10 months; median PFS, 5 months) (OS and PFS: p < 0.05, log-rank test). Our results are consistent with those of a previous study that suggested that PTX + RT followed by maintenance taxane chemotherapy is a better therapeutic option than other interventions, including surgery.
  • Super-resolution imaging detects BP180 autoantigen in immunoglobulin M pemphigoid.
    Yoko Hirano, Hiroaki Iwata, Masumi Tsujuwaki, Shoko Mai, Yosuke Mai, Keisuke Imafuku, Kentaro Izumi, Hiroshi Koga, Hideyuki Ujiie
    The Journal of dermatology, 49, 3, 374, 378, Mar. 2022, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal, Bullous pemphigoid is generally caused by immunoglobulin (Ig)G autoantibodies against hemidesmosomal BP180 and/or BP230. Recently, the concept of IgM pemphigoid has been proposed. A 23-year-old Japanese woman presented with a 4-month history of severely itchy papules showing subepidermal separations with mild neutrophil infiltration. Direct immunofluorescence (DIF) revealed IgM deposits at the dermoepidermal junction, but neither IgG nor IgA deposits. Indirect immunofluorescence on 1 M NaCl-split skin demonstrated deposits on the epidermal side. The optical density (OD) value of a modified IgM enzyme-linked immunosorbent assay for full-length BP180, but not for BP180-NC16A, was increased. The patient was diagnosed with IgM pemphigoid and was treated with diphenyl sulfone at 50 mg/day without recurrence. To confirm the precise autoantigen, we tried to obtain super-resolution imaging. The deposition pattern of IgM autoantibodies seemed to be oriented parallel to that of BP180. The detailed images detect DIF deposits apart from BP180-NC16A staining, but are close to type VII collagen-NC1 staining. This result suggests that the IgM autoantibodies in the patient might target the C-terminus of BP180. IgM pemphigoid is still not a widely accepted concept, and the clinical course remains unknown. We will carefully follow-up the patient. Super-resolution images may help to detect precise autoantigens in autoimmune blistering diseases.
  • IgA nephropathy preceded by erythroderma with eosinophilia.
    Mina Takatsu, Ken Natsuga, Fumihiko Hattanda, Hideyuki Ujiie
    European journal of dermatology : EJD, 15 Feb. 2022, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • A toe ulcer in a patient with essential thrombocythemia successfully treated with a combination of anagrelide and prednisolone.
    Tatsuro Sugai, Yasuyuki Yamaguchi, Atsushi Narahira, Takuma Nohara, Hiroyuki Nakamura, Ko Ebata, Hideyuki Ujiie
    Dermatologic therapy, 35, 2, e15221, Feb. 2022, [International Magazine]
    English
  • Cyclin-dependent kinase 4/6 inhibitors suppress tumor growth in extramammary Paget’s disease
    Shinya Kitamura, Teruki Yanagi, Takuya Maeda, Hideyuki Ujiie
    Cancer Science, 113, 2, 802, 807, Feb. 2022, [International Magazine]
    English, Scientific journal
  • IgA nephropathy preceded by erythroderma with eosinophilia.
    Mina Takatsu, Ken Natsuga, Fumihiko Hattanda, Hideyuki Ujiie
    European journal of dermatology : EJD, 32, 1, 127, 129, 01 Jan. 2022, [International Magazine]
    English, Scientific journal
  • Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases.
    Hideyuki Ujiie, David Rosmarin, Michael P Schön, Sonja Ständer, Katharina Boch, Martin Metz, Marcus Maurer, Diamant Thaci, Enno Schmidt, Connor Cole, Kyle T Amber, Dario Didona, Michael Hertl, Andreas Recke, Hanna Graßhoff, Alexander Hackel, Anja Schumann, Gabriela Riemekasten, Katja Bieber, Gant Sprow, Joshua Dan, Detlef Zillikens, Tanya Sezin, Angela M Christiano, Kerstin Wolk, Robert Sabat, Khalaf Kridin, Victoria P Werth, Ralf J Ludwig
    Frontiers in medicine, 9, 875492, 875492, 2022, [International Magazine]
    English, Scientific journal, An estimated 20-25% of the population is affected by chronic, non-communicable inflammatory skin diseases. Chronic skin inflammation has many causes. Among the most frequent chronic inflammatory skin diseases are atopic dermatitis, psoriasis, urticaria, lichen planus, and hidradenitis suppurativa, driven by a complex interplay of genetics and environmental factors. Autoimmunity is another important cause of chronic skin inflammation. The autoimmune response may be mainly T cell driven, such as in alopecia areata or vitiligo, or B cell driven in chronic spontaneous urticaria, pemphigus and pemphigoid diseases. Rare causes of chronic skin inflammation are autoinflammatory diseases, or rheumatic diseases, such as cutaneous lupus erythematosus or dermatomyositis. Whilst we have seen a significant improvement in diagnosis and treatment, several challenges remain. Especially for rarer causes of chronic skin inflammation, early diagnosis is often missed because of low awareness and lack of diagnostics. Systemic immunosuppression is the treatment of choice for almost all of these diseases. Adverse events due to immunosuppression, insufficient therapeutic responses and relapses remain a challenge. For atopic dermatitis and psoriasis, a broad spectrum of innovative treatments has been developed. However, treatment responses cannot be predicted so far. Hence, development of (bio)markers allowing selection of specific medications for individual patients is needed. Given the encouraging developments during the past years, we envision that many of these challenges in the diagnosis and treatment of chronic inflammatory skin diseases will be thoroughly addressed in the future.
  • The relevance of complement in pemphigoid diseases: A critical appraisal.
    Cristian Papara, Christian M Karsten, Hideyuki Ujiie, Enno Schmidt, Leon F Schmidt-Jiménez, Adrian Baican, Patricia C Freire, Kentaro Izumi, Katja Bieber, Matthias Peipp, Admar Verschoor, Ralf J Ludwig, Jörg Köhl, Detlef Zillikens, Christoph M Hammers
    Frontiers in immunology, 13, 973702, 973702, 2022, [International Magazine]
    English, Scientific journal, Pemphigoid diseases are autoimmune chronic inflammatory skin diseases, which are characterized by blistering of the skin and/or mucous membranes, and circulating and tissue-bound autoantibodies. The well-established pathomechanisms comprise autoantibodies targeting various structural proteins located at the dermal-epidermal junction, leading to complement factor binding and activation. Several effector cells are thus attracted and activated, which in turn inflict characteristic tissue damage and subepidermal blistering. Moreover, the detection of linear complement deposits in the skin is a diagnostic hallmark of all pemphigoid diseases. However, recent studies showed that blistering might also occur independently of complement. This review reassesses the importance of complement in pemphigoid diseases based on current research by contrasting and contextualizing data from in vitro, murine and human studies.
  • Case report: Difference in outcomes between two cases of Hailey-Hailey disease treated with apremilast.
    Misako Yamaga, Toshinari Miyauchi, Jin Teng Peh, Sota Itamoto, Yosuke Mai, Hiroaki Iwata, Toshifumi Nomura, Hideyuki Ujiie
    Frontiers in genetics, 13, 884359, 884359, 2022, [International Magazine]
    English, Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis clinically characterized by recurrent erythematous plaques and erosions mainly on the intertriginous regions. Although HHD seriously affects quality of life, conventional treatments often fail to provide long-term relief for most patients. The effectiveness of apremilast, a phosphodiesterase-4 inhibitor, against severe HHD was first reported in 2018, and after further testing, this agent is currently expected to be established as an efficacious and safe therapeutic option. Here we report two cases of HHD treated with apremilast which showed opposite outcomes. Although the case with extremely severe symptoms showed remarkable and long-lasting improvement with apremilast used after acute treatment with oral corticosteroid, the other case, with milder symptoms treated only with apremilast, showed no improvement. Our transcriptome analysis using skin samples collected prior to apremilast administration revealed the involvement of the NF-κB signaling pathway, which is related to the responses to bacteria and other organisms. However, this pathway was more strongly activated in case 2 than in case 1, suggesting that the steroid treatment preceding apremilast may have been effective and supportive in the apremilast-responding case. One of the two cases highlights the potential of apremilast as a treatment option for HHD, but the other underlines the difficulties in managing HHD and the complexity of the disease background. The accumulation of cases and larger clinical studies are expected to precisely evaluate the safety and efficacy of apremilast, and the potential for therapies in combination with conventional treatments.
  • Case of hereditary leiomyomatosis and renal cell cancer showing multiple cutaneous leiomyomas harboring a recurrent nonsense mutation in the fumarate hydratase gene.
    Shoko Mai, Teruki Yanagi, Mayuna Shimano, Wataru Nishie, Ken Arita, Hideyuki Ujiie
    The Journal of dermatology, 49, 1, e42-e43, Jan. 2022, [International Magazine]
    English
  • The significance of preclinical anti-BP180 autoantibodies.
    Yosuke Mai, Kentaro Izumi, Shoko Mai, Hideyuki Ujiie
    Frontiers in immunology, 13, 963401, 963401, 2022, [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease. Although the pathomechanism of BP onset has yet to be elucidated in detail, BP autoantibodies targeting two hemidesmosomal components, BP180 and BP230, are known to play a pivotal role in BP pathogenesis. Thus, the detection and measurement of BP autoantibodies are necessary for diagnosing BP and monitoring the disease activity. Immune assays such as immunofluorescence microscopy, immunoblotting, and ELISAs using BP180 and BP230 detect BP autoantibodies in most BP cases with high specificity; however, BP autoantibodies are sometimes detected in BP patients before the onset of this disease. BP autoantibodies that are detected in patients without typical tense blisters are defined as "preclinical BP autoantibodies". These preclinical BP autoantibodies are detected even in a low percentage of normal healthy individuals. Although the importance of preclinical BP autoantibodies remains elusive, these autoantibodies might be a potential risk factor for subsequent BP development. Therefore, previous comparative epidemiological studies have focused on the prevalence of preclinical BP autoantibodies in populations susceptible to BP (e.g., the elderly) or in diseases with a higher risk of comorbid BP. This mini-review summarizes the literature on the prevalence of preclinical BP autoantibodies in patients with various conditions and diseases, and we discuss the significance of preclinical BP autoantibody detection.
  • IgA nephropathy preceded by erythroderma with eosinophilia.
    Mina Takatsu, Ken Natsuga, Fumihiko Hattanda, Hideyuki Ujiie
    European journal of dermatology : EJD, 32, 1, 127, 129, 01 Jan. 2022, [International Magazine]
    English, Scientific journal
  • Granzyme B inhibition reduces disease severity in autoimmune blistering diseases
    Sho Hiroyasu, Matthew R. Zeglinski, Hongyan Zhao, Megan A. Pawluk, Christopher T. Turner, Anika Kasprick, Chiharu Tateishi, Wataru Nishie, Angela Burleigh, Peter A. Lennox, Nancy Van Laeken, Nick J. Carr, Frank Petersen, Richard I. Crawford, Hiroshi Shimizu, Daisuke Tsuruta, Ralf J. Ludwig, David J. Granville
    Nature Communications, 12, 1, Springer Science and Business Media LLC, Dec. 2021
    Scientific journal, AbstractPemphigoid diseases refer to a group of severe autoimmune skin blistering diseases characterized by subepidermal blistering and loss of dermal-epidermal adhesion induced by autoantibody and immune cell infiltrate at the dermal-epidermal junction and upper dermis. Here, we explore the role of the immune cell-secreted serine protease, granzyme B, in pemphigoid disease pathogenesis using three independent murine models. In all models, granzyme B knockout or topical pharmacological inhibition significantly reduces total blistering area compared to controls. In vivo and in vitro studies show that granzyme B contributes to blistering by degrading key anchoring proteins in the dermal-epidermal junction that are necessary for dermal-epidermal adhesion. Further, granzyme B mediates IL-8/macrophage inflammatory protein-2 secretion, lesional neutrophil infiltration, and lesional neutrophil elastase activity. Clinically, granzyme B is elevated and abundant in human pemphigoid disease blister fluids and lesional skin. Collectively, granzyme B is a potential therapeutic target in pemphigoid diseases.
  • The pathogeneses of pemphigus and pemphigoid diseases.
    Hideyuki Ujiie, Jun Yamagami, Hayato Takahashi, Kentaro Izumi, Hiroaki Iwata, Gang Wang, Daisuke Sawamura, Masayuki Amagai, Detlef Zillikens
    Journal of dermatological science, 104, 3, 154, 163, Dec. 2021, [International Magazine]
    English, Scientific journal, Autoimmune bullous diseases (AIBDs) are skin disorders which are mainly induced by autoantibodies against desmosomal or hemidesmosomal structural proteins. Previous studies using patients' samples and animal disease models identified target antigens and elucidated the mechanisms of blister formation. Pemphigus has been the subject of more active clinical and basic research than any other AIBD. These efforts have revealed the pathogenesis of pemphigus, which in turn has led to optimal diagnostic methods and novel therapies, such as rituximab. In bullous pemphigoid (BP), studies with passive-transfer mouse models using rabbit anti-mouse BP180 antibodies and studies with passive-transfer or active mouse models using autoantigen-humanized mice elucidated the immune reactions to BP180 in vivo. Recently, dipeptidyl peptidase-4 inhibitors have attracted attention as a trigger for BP. For epidermolysis bullosa acquisita (EBA), investigations using mouse models are actively under way and several molecules have been identified as targets for novel therapies. In this review, we give an overview and discussion of the recent progress in our understanding of the pathogenesis of pemphigus, BP, and EBA. Further studies on the breakdown of self-tolerance and on the identification of key molecules that are relevant to blister formation may expand our understanding of the etiology of AIBDs and lead to the development of novel therapeutic strategies.
  • Development of Verruca Plana from Human Papillomavirus 78 Dependent on Host Immune State.
    Mayuna Shimano, Toshinari Miyauchi, Teruki Yanagi, Toshifumi Nomura, Hideyuki Ujiie
    Acta dermato-venereologica, 101, 11, adv00608, 30 Nov. 2021, [International Magazine]
    English, Scientific journal
  • Unilateral Naevoid Telangiectasia Associated with Oral Contraceptive.
    Takuma Nohara, Ken Natsuga, Atsushi Yasuoka, Hideyuki Ujiie
    Acta dermato-venereologica, 101, 11, adv00595, 17 Nov. 2021, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • Macrolide/fluoroquinolone-resistant Campylobacter jejuni-induced cellulitis in a patient with X-linked agammaglobulinaemia successfully treated with carbapenem.
    Joohyung Youh, Keisuke Imafuku, Teruki Yanagi, Sho Nakakubo, Yosuke Mai, Hideyuki Kosumi, Takuya Kawamura, Ken Muramatsu, Chihiro Shiiya, Ayame Kimura, Sota Itamoto, Masafumi Yamada, Hideyuki Ujiie
    Dermatologic therapy, 34, 6, e15176, Nov. 2021, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • Loss of FAM83H promotes cell migration and invasion in cutaneous squamous cell carcinoma via impaired keratin distribution.
    Keiko Tokuchi, Shinya Kitamura, Takuya Maeda, Masashi Watanabe, Shigetsugu Hatakeyama, Satoshi Kano, Shinya Tanaka, Hideyuki Ujiie, Teruki Yanagi
    Journal of dermatological science, 104, 2, 112, 121, Nov. 2021, [Peer-reviewed], [International Magazine]
    English, Scientific journal, BACKGROUNDS: FAM83H is essential for amelogenesis, but recent reports implicate that FAM83H is involved in the tumorigenesis. We previously clarified that TRIM29 binds to FAM83H to regulate keratin distribution and squamous cell migration. However, little is known about FAM83H in normal/malignant skin keratinocytes. OBJECTIVE: To investigate the expression of FAM83H in cutaneous squamous cell carcinoma (SCC) and its physiological function. METHODS: Immunohistochemical analysis and RT-PCR of human SCC tissues were performed. Next, we examined the effect of FAM83H knockdown/overexpression in SCC cell lines using cell proliferation, migration, and invasion assay. To investigate the molecular mechanism, immunoprecipitation of FAM83H was examined. Further, Immunofluorescence staining was performed. Finally, we examined the correlation between the expressions of FAM83H and the keratin distribution. RESULTS: FAM83H expression was lower in SCC lesions than in normal epidermis and correlated with differentiation grade. The mRNA expression levels of FAM83H in SCC tumors were also lower than in normal epidermis. The knockdown of FAM83H enhanced SCC cell migration and invasion, whereas the overexpression of FAM83H led to decreases in both. Furthermore, the knockdown of FAM83H enhanced the cancer cell metastasis in vivo. FAM83H formed a complex with TRIM29 and keratins. The knockdown of FAM83H altered keratin distribution and solubility. Clinically, the loss of FAM83H correlates with an altered keratin distribution. CONCLUSION: Our findings reveal a critical function for FAM83H in regulating keratin distribution, as well as in the migration/invasion of cutaneous SCC, suggesting that FAM83H could be a crucial molecule in the tumorigenesis of cutaneous SCC.
  • Hidradenitis suppurativa successfully treated with two-stage surgery under disease control with adalimumab.
    Akihiro Orita, Shinya Kitamura, Takuya Maeda, Teruki Yanagi, Hiroaki Iwata, Keiko Shiba-Tokuchi, Toshifumi Nomura, Hideyuki Ujiie
    The Journal of dermatology, 49, 1, e1-e2, 10 Sep. 2021, [Peer-reviewed], [Last author], [International Magazine]
    English
  • Possible role of epiregulin from dermal fibroblasts in the keratinocyte hyperproliferation of psoriasis.
    Hiroaki Iwata, Naoya Haga, Hideyuki Ujiie
    The Journal of dermatology, 48, 9, 1433, 1438, Sep. 2021, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal, Psoriasis, an immune-mediated inflammatory disease, is characterized by keratinocyte hyperproliferation. Tumor necrosis factor (TNF)-α, interleukin (IL)-23, and IL-17A play critical roles in the pathogenesis of psoriasis. IL-17A secreted by T-helper 17 acts more directly against keratinocytes than TNF-α or IL-23 do. Regarding the receptors of cytokines, fibroblasts also express receptors against IL-17A and TNF-α, and induce the production of growth factors. Epiregulin (EREG), an epidermal growth factor receptor ligand, is produced by both keratinocytes and fibroblasts. EREG enhances keratinocyte proliferation and differentiation. We hypothesized that fibroblasts stimulated with IL-17A and/or TNF-α may play a role in epidermal hyperproliferation through the production of epidermal growth factors in psoriasis. The mRNA expression of EREG was found to be significantly upregulated by co-stimulation with IL-17A and TNF-α (mean, 49.2-fold). Furthermore, the stimulation with TNF-α alone, but not IL-17A alone, induced significant increases. Immunofluorescent staining demonstrated that the protein expression level of EREG was also increased in fibroblasts stimulated with these cytokines. Stimulation with EREG significantly enhanced keratinocyte proliferation in vitro. In human psoriatic patients' skin, immunofluorescence staining of EREG showed significantly high intensity in the dermis of lesional skin. In conclusion, cytokine stimulation with TNF-α and IL-17A induces the overexpression of EREG from dermal fibroblasts in the lesional skin of psoriasis, and plays a role in epidermal hyperproliferation.
  • X連鎖無γグロブリン血症患者に発症した多剤耐性Campylobacter jejuniによる蜂窩織炎の1例               
    劉 柱亨, 柳 輝希, 板本 想太, 木村 彩萌, 眞井 洋輔, 村松 憲, 今福 恵輔, 氏家 英之, 中久保 祥, 山田 雅文
    日本皮膚科学会雑誌, 131, 9, 2072, 2072, (公社)日本皮膚科学会, Aug. 2021
    Japanese
  • Pigmented eccrine poroma on the palm mimicking seborrheic keratosis: Dermoscopic findings and a review of reported cases.
    Mayuna Shimano, Shinya Kitamura, Takuya Maeda, Emi Takakuwa, Teruki Yanagi, Hideyuki Ujiie
    The Australasian journal of dermatology, 62, 3, e477-e479, Aug. 2021, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • A multicenter, open-label, uncontrolled, single-arm phase 2 study of tirabrutinib, an oral Bruton's tyrosine kinase inhibitor, in pemphigus.
    Jun Yamagami, Hideyuki Ujiie, Yumi Aoyama, Norito Ishii, Chiharu Tateishi, Akira Ishiko, Tomoki Ichijima, Shunsuke Hagihara, Koji Hashimoto, Masayuki Amagai
    Journal of dermatological science, 103, 3, 135, 142, 06 Jul. 2021, [Peer-reviewed], [International Magazine]
    English, Scientific journal, BACKGROUND: The treatment of pemphigus is based on systemic corticosteroid use and adjuvant therapies, but some patients are resistant to conventional therapy. Tirabrutinib is a highly selective oral Bruton's tyrosine kinase inhibitor that may be clinically effective in treating pemphigus by suppressing B-cell signaling. OBJECTIVE: We investigated the efficacy and safety of tirabrutinib in patients with refractory pemphigus. METHODS: This was a multicenter, open-label, single-arm phase 2 study of Japanese patients with refractory pemphigus receiving appropriate treatment with an oral corticosteroid and adjuvant therapies. Patients received postprandial oral tirabrutinib 80 mg once daily for 52 weeks. After 16 weeks of tirabrutinib treatment, the corticosteroid dose was tapered to ≤10 mg/day of prednisolone equivalent. RESULTS: In total, 16 patients were evaluated (mean age, 52.5 years; 50 % male). The complete remission rate after 24 weeks of treatment (primary endpoint) was 18.8 % (3/16; 95 % confidence interval, 6.6 %-43.0 %). By Week 52, eight patients (50.0 %) achieved complete remission and 10 patients (62.5 %) achieved remission. Over 52 weeks of treatment, the mean prednisolone dose decreased from 17.03 to 7.65 mg/day. Incidences of adverse events (AEs) and adverse drug reactions were 87.5 % and 43.8 %, respectively. A relationship with tirabrutinib was ruled out for all serious AEs and Grade ≥3 AEs. CONCLUSION: Treatment with tirabrutinib enabled remission and reduced oral corticosteroid exposure over time and did not result in any major safety concerns in patients with refractory pemphigus. Thus, oral tirabrutinib may be a new treatment option for patients with refractory pemphigus.
  • Hair follicle stem cell progeny heal blisters while pausing skin development.
    Yu Fujimura, Mika Watanabe, Kota Ohno, Yasuaki Kobayashi, Shota Takashima, Hideki Nakamura, Hideyuki Kosumi, Yunan Wang, Yosuke Mai, Andrea Lauria, Valentina Proserpio, Hideyuki Ujiie, Hiroaki Iwata, Wataru Nishie, Masaharu Nagayama, Salvatore Oliviero, Giacomo Donati, Hiroshi Shimizu, Ken Natsuga
    EMBO reports, 22, 7, e50882, 05 Jul. 2021, [Peer-reviewed], [Internationally co-authored], [International Magazine]
    English, Scientific journal, Injury in adult tissue generally reactivates developmental programs to foster regeneration, but it is not known whether this paradigm applies to growing tissue. Here, by employing blisters, we show that epidermal wounds heal at the expense of skin development. The regenerated epidermis suppresses the expression of tissue morphogenesis genes accompanied by delayed hair follicle (HF) growth. Lineage tracing experiments, cell proliferation dynamics, and mathematical modeling reveal that the progeny of HF junctional zone stem cells, which undergo a morphological transformation, repair the blisters while not promoting HF development. In contrast, the contribution of interfollicular stem cell progeny to blister healing is small. These findings demonstrate that HF development can be sacrificed for the sake of epidermal wound regeneration. Our study elucidates the key cellular mechanism of wound healing in skin blistering diseases.
  • Dermoscopic features of sebaceous carcinoma of the nipple.
    Takuya Kawamura, Shinya Kitamura, Takuya Maeda, Akihiro Orita, Teruki Yanagi, Hideyuki Ujiie
    The Journal of dermatology, 48, 7, e327-e328, Jul. 2021, [Peer-reviewed], [Last author], [International Magazine]
    English, Scientific journal
  • Spindle cell lipoma with a palisading pattern: A case report.
    Mayuna Shimano, Shinya Kitamura, Takuya Maeda, Teruki Yanagi, Emi Takakuwa, Hideyuki Ujiie
    The Journal of dermatology, 48, 7, e351-e352, Jul. 2021, [Peer-reviewed], [International Magazine]
    English
  • A case of non-bullous pemphigoid induced by IgG4 autoantibodies targeting BP230.
    N Yoshimoto, S Takashima, T Kawamura, E Inamura, T Sugai, I Ujiie, K Izumi, K Natsuga, W Nishie, H Shimizu, H Ujiie
    Journal of the European Academy of Dermatology and Venereology : JEADV, 35, 4, e282-e285, Apr. 2021, [Peer-reviewed], [Last author, Corresponding author], [International Magazine]
    English, Scientific journal
  • Clinical characteristics and outcomes of bullous pemphigoid patients with versus without oral prednisolone treatment.
    Inkin Ujiie, Hiroaki Iwata, Norihiro Yoshimoto, Kentaro Izumi, Hiroshi Shimizu, Hideyuki Ujiie
    The Journal of dermatology, 48, 4, 502, 510, Apr. 2021, [Peer-reviewed], [Last author, Corresponding author], [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP) varies in severity and stratified treatments are needed. However, there are no definitive standards for choosing appropriate treatments. To elucidate the factors involved in choosing treatments and the clinical outcomes of BP, we retrospectively reviewed the clinical records of 78 BP patients at a single center. Of the 78 patients, 49 (62.8%) were treated with oral prednisolone (PSL) and 29 (37.2%) were treated without PSL. The patients with older age, lower Bullous Pemphigoid Disease Area Index (BPDAI), and/or lower anti-BP180NC16a antibody titer at onset tended to be treated without oral PSL. Notably, only 9.1% patients without PSL experienced relapse, whereas 36.7% patients with oral PSL experienced relapse when the PSL was around 0.1 mg/kg. It suggests that the patients with mild disease severity could be well controlled without oral PSL. Receiver-operator curve analysis demonstrated that the cut-off value for the use of oral PSL was 31 for total BPDAI and was 7 for BPDAI skin urticaria/erythema, with a high (>0.9) area under the curve. Notably, none of the patients who were negative for the anti-BP180NC16a antibody at onset experienced relapse even though they were treated without PSL. In conclusion, in BP patients who were negative for anti-BP180NC16a antibody at onset, with a total BPDAI score of less than 31 or with an urticaria/erythema score of less than 7 can be treated without PSL. When the PSL is tapered to around 0.1 mg/kg, we should carefully monitor the patients to detect relapse.
  • A case of mucous membrane pemphigoid with anti-BP230 autoantibodies alone.
    Norihiro Yoshimoto, Inkin Ujiie, Emi Inamura, Ken Natsuga, Wataru Nishie, Hiroshi Shimizu, Hideyuki Ujiie
    International journal of dermatology, 60, 3, e92-e94, Mar. 2021, [Peer-reviewed], [Last author, Corresponding author], [International Magazine]
    English, Scientific journal
  • [Not Available].
    Emi Inamura, Masumi Tsujiwaki, Hideyuki Ujiie, Wataru Nishie, Hiroo Hata, Hiroshi Shimizu, Hiroaki Iwata
    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 19, 1, 105, 108, Jan. 2021, [International Magazine]
    German
  • TRIM29 in Cutaneous Squamous Cell Carcinoma.
    Che-Yuan Hsu, Teruki Yanagi, Hideyuki Ujiie
    Frontiers in medicine, 8, 804166, 804166, 2021, [International Magazine]
    English, Scientific journal, Tripartite motif (TRIM) proteins play important roles in a wide range of cell physiological processes, such as signal transduction, transcriptional regulation, innate immunity, and programmed cell death. TRIM29 protein, encoded by the ATDC gene, belongs to the RING-less group of TRIM protein family members. It consists of four zinc finger motifs in a B-box domain and a coiled-coil domain, and makes use of the B-box domain as E3 ubiquitin ligase in place of the RING. TRIM29 was found to be involved in the formation of homodimers and heterodimers in relation to DNA binding; additional studies have also demonstrated its role in carcinogenesis, DNA damage signaling, and the suppression of radiosensitivity. Recently, we reported that TRIM29 interacts with keratins and FAM83H to regulate keratin distribution. Further, in cutaneous SCC, the expression of TRIM29 is silenced by DNA methylation, leading to the loss of TRIM29 and promotion of keratinocyte migration. This paper reviews the role of TRIM family proteins in malignant tumors, especially the role of TRIM29 in cutaneous SCC.
  • Bullous pemphigoid associated with psoriasis showing marked neutrophilic infiltrates.
    Emi Inamura, Masumi Tsujiwaki, Hideyuki Ujiie, Wataru Nishie, Hiroo Hata, Hiroshi Shimizu, Hiroaki Iwata
    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 19, 1, 105, 108, Jan. 2021, [Peer-reviewed], [International Magazine]
    English
  • Regulatory T cell subsets in bullous pemphigoid and dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid.
    Ken Muramatsu, Miao Zheng, Norihiro Yoshimoto, Takamasa Ito, Inkin Ujiie, Hiroaki Iwata, Hiroshi Shimizu, Hideyuki Ujiie
    Journal of dermatological science, 100, 1, 23, 30, Oct. 2020, [Peer-reviewed], [Last author, Corresponding author], [International Magazine]
    English, Scientific journal, BACKGROUND: Regulatory T (Treg) cells play an essential role in peripheral immune tolerance. Bullous pemphigoid (BP) is the most common blistering disease and is caused by autoantibodies to two BP antigens: type XVII collagen and BP230. Recently, we reported that Treg cell dysfunction may cause the production of autoantibodies to BP antigens. Several studies have suggested an association between Treg cells and BP pathogenesis. However, Treg cells are heterogeneous in humans, leading to inconsistent results in previous studies. OBJECTIVE: To assess functional Treg subsets in BP. METHODS: We examined three distinct Treg subsets in conventional BP (cBP) patients before versus after systemic corticosteroid treatment, dipeptidyl peptidase-4 inhibitor-associated BP (DPP-4i-BP) patients, younger controls and older controls. RESULTS: We found that total Treg cells and all Treg cell subsets were increased in cBP patients before treatment and decreased by systemic corticosteroid treatment. In contrast, neither total Treg cells nor all Treg subsets were increased in DPP-4i-BP. Notably, CD45RA- Foxp3hi effector Treg cells positively correlated with disease severity in cBP, whereas CD45RA+Foxp3lo naïve Treg cells positively correlated with the disease severity in DPP-4i-BP. CONCLUSION: These findings suggest that Treg cells are differently involved in the pathogeneses of cBP and DPP-4i-BP.
  • Case of bullous erythema ab igne mimicking localized bullous pemphigoid.
    Naoya Haga, Hideyuki Ujiie, Takamasa Ito, Yasuyuki Yamaguchi, Takuya Mizukami, Sho Katayama, Hiroshi Shimizu
    The Journal of dermatology, 47, 8, e288-e289, Aug. 2020, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal
  • Epitope spreading possibly from BP230 to the NC16A domain of BP180 preceding disease progression in bullous pemphigoid.
    Takashi Seo, Hideyuki Ujiie, Inkin Ujiie, Hiroaki Iwata, Hiroshi Shimizu
    The Journal of dermatology, 47, 7, e255-e257, Jul. 2020, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal
  • Prevalence of infectious diseases in patients with autoimmune blistering diseases.
    Inkin Ujiie, Hideyuki Ujiie, Norihiro Yoshimoto, Hiroaki Iwata, Hiroshi Shimizu
    The Journal of dermatology, 47, 4, 378, 384, Apr. 2020, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal, A long-term immunosuppressive treatment can provoke latent infections. Autoimmune blistering diseases (AIBD) are mostly treated with systemic immunosuppressive agents. To prevent the reactivation or exacerbation of existing latent infections, patients must be screened for infectious diseases before immunosuppressive treatments are initiated. However, the prevalence of infectious diseases in AIBD remains to be elucidated. To evaluate the necessity of screening infectious diseases in AIBD, we retrospectively reviewed the clinical records of 215 patients at a single center with AIBD for hepatitis B virus (HBV), hepatitis C virus (HCV), Mycobacterium tuberculosis, Treponema pallidum, human T-cell leukemia virus type 1 (HTLV-1) and HIV infections. Approximately 40% of patients were infected with HBV. During systemic corticosteroid treatment, HBV DNA became positive in 3.4% of cases. Antibodies to HCV, interferon-γ release assays for M. tuberculosis and the T. pallidum latex agglutination test were positive in 0.6%, 6.6% and 1.2% cases, respectively. Neither HTLV-1 nor HIV infections were detected. In conclusion, checks for HBV and M. tuberculosis infections should be made before immunosuppressive treatments are started, because of the high prevalence of these potentially life-threatening infections. Other infections should be tested for depending on the patient's risk factors.
  • Rosai-Dorfman disease: earlier spontaneous regression of skin lesions relative to nasal, pharyngeal, and bone lesions.
    Kazumasa Sato, Hideyuki Ujiie, Shinichi Nakazato, Mika Watanabe, Erika Watanabe, Teruki Yanagi, Yuji Nakamaru, Dai Takagi, Ryuta Arai, Tomohiro Onodera, Takeshi Kondo, Takanori Teshima, Hiroshi Shimizu
    European journal of dermatology : EJD, 30, 2, 182, 183, 01 Apr. 2020, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal
  • Linear IgA/IgG bullous dermatosis with autoantibodies directing the native and processed forms of BP180.
    E Inamura, W Nishie, Y Yamaguchi, Y Fujimura, H Ujiie, K Natsuga, H Shimizu
    The British journal of dermatology, 182, 4, 1061, 1062, Apr. 2020, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • 水疱性類天疱瘡との鑑別を要した温熱性紅斑および低温熱傷の1例               
    羽賀 直哉, 氏家 英之, 伊東 孝政, 山口 泰之, 水上 卓哉, 片山 奨, 清水 宏
    日本皮膚科学会雑誌, 130, 3, 392, 393, (公社)日本皮膚科学会, Mar. 2020
    Japanese
  • Localized bullous pemphigoid recurring at different sites: two case reports.
    Akito Hasegawa, Satoru Shinkuma, Tatsuya Katsumi, Naomi Kasahara, Kaoru Ito, Hideyuki Ujiie, Norito Ishii, Takashi Hashimoto, Riichiro Abe
    European journal of dermatology : EJD, 06 Feb. 2020, [Peer-reviewed], [International Magazine]
    English, Scientific journal, BACKGROUND: Localized bullous pemphigoid is a relatively rare variant of bullous pemphigoid. Lesions develop only in localized sites including the legs or palms and soles and occasionally appear in trauma-affected body parts. In some cases, the skin lesions spread to the entire body, while in others, they remain localized and improve spontaneously or with treatment using topical corticosteroids. Rarely, the lesions recur at sites different from those of the original lesions, after the initial lesions have completely healed. OBJECTIVES: To investigate the clinical course of patients with localized bullous pemphigoid. MATERIALS AND METHODS: Two cases of localized bullous pemphigoid that recurred at sites distant from those of the initial lesions were reported. RESULTS: Case 1 involved a 62-year-old woman with mucous membrane pemphigoid. One year after the improvement of mucosal symptoms, new lesions appeared in the periumbilical area. The lesions resolved after topical corticosteroid treatment. This case is the first report of localized bullous pemphigoid occurring after an improvement of mucous membrane pemphigoid. Case 2 involved an 81-year-old man who bruised his right lower leg and developed erythematous plaques and tense bullae. The patient was diagnosed with localized bullous pemphigoid and was treated with topical corticosteroid. However, six months later, new lesions appeared on the palms and soles. The patient responded well to oral prednisolone. CONCLUSIONS: Since localized bullous pemphigoid may have a variable clinical course, clinicians should observe affected patients carefully over a long period of time.
  • A case of epidermolysis bullosa acquisita with ocular involvement
    Mami Murata, Nobuyuki Asano, Hideyuki Ujiie, Takahiro Yamada, Yutaka Shimomura
    Nishinihon Journal of Dermatology, 81, 6, 478, 482, Western Division of Japanese Dermatological Association, 01 Dec. 2019
    Japanese, Scientific journal
  • Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita).
    Hideyuki Ujiie, Hiroaki Iwata, Jun Yamagami, Takekuni Nakama, Yumi Aoyama, Shigaku Ikeda, Norito Ishii, Keiji Iwatsuki, Michiko Kurosawa, Daisuke Sawamura, Akiko Tanikawa, Daisuke Tsuruta, Wataru Nishie, Wataru Fujimoto, Masayuki Amagai, Hiroshi Shimizu
    The Journal of dermatology, 46, 12, 1102, 1135, Dec. 2019, [Invited], [Lead author, Corresponding author], [International Magazine]
    English, Scientific journal, The pemphigoid group is a category of autoimmune subepidermal blistering diseases in which autoantibodies deposit linearly at the epidermal basement membrane zone (BMZ). The main subtypes of pemphigoid mediated by immunoglobulin G autoantibodies are bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). To establish the first guidelines approved by the Japanese Dermatological Association for the management of pemphigoid diseases, the Committee for Guidelines for the Management of Pemphigoid Diseases (Including EBA) was founded as part of the Study Group for Rare Intractable Skin Diseases under the Ministry of Health, Labor and Welfare Research Project on Overcoming Intractable Diseases. These guidelines aim to provide current information for the management of BP, MMP and EBA in Japan. Based on evidence, the guidelines summarize the clinical and immunological manifestations, pathophysiologies, diagnostic criteria, disease severity determination criteria, treatment algorithms and treatment recommendations. Because of the rarity of these diseases, there are few clinical studies with a high degree of evidence, so several parts of these guidelines were established based on the opinions of the Committee. To further optimize these guidelines, periodic revision in line with the new evidence is necessary.
  • [Not Available].
    Satoko Shimizu, Yuka Takashima, Yuka Maya, Fumihiro Kodama, Mayumi Yokozeki, Koki Tomizawa, Ken Muramatsu, Hideyuki Ujiie, Hiroshi Shimizu, Reine Moriuchi
    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 17, 10, 1066, 1068, Oct. 2019, [International Magazine]
    German
  • Disseminated Mycobacterium intracellulare infection that led to recognition of idiopathic CD4+ lymphocytopenia.
    Satoko Shimizu, Yuka Takashima, Yuka Maya, Fumihiro Kodama, Mayumi Yokozeki, Koki Tomizawa, Ken Muramatsu, Hideyuki Ujiie, Hiroshi Shimizu, Reine Moriuchi
    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 17, 10, 1066, 1068, Oct. 2019, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • 発症から診断までに10年以上が経過した粘膜類天疱瘡の1例               
    鈴木 丈雄, 新熊 悟, 荻根沢 真帆子, 会沢 敦子, 高橋 奈央, 泉 健太郎, 氏家 英之, 西江 渉, 阿部 理一郎
    臨床皮膚科, 73, 10, 813, 817, (株)医学書院, Sep. 2019, [Peer-reviewed]
    Japanese
  • Clinical and immunological features of pemphigus relapse.
    I Ujiie, H Ujiie, H Iwata, H Shimizu
    The British journal of dermatology, 180, 6, 1498, 1505, Jun. 2019, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal, BACKGROUND: More than half of patients with pemphigus experience relapse during the disease course. The risk factors and clinical and immunological characteristics of relapse remain largely unclear. OBJECTIVES: To elucidate the risk factors and clinical features of pemphigus relapse. METHODS: We carried out a retrospective review of the clinical records of 42 cases of pemphigus at a single centre. RESULTS: Sixty-two per cent of patients experienced relapse, usually when oral prednisolone was tapered to around 0·1 mg kg-1 . In mucocutaneous pemphigus vulgaris (mcPV), the initial doses (mean ± SD) of prednisolone were significantly lower in patients with relapse (0·78 ± 0·24 mg kg-1 ) than in those without relapse (1·01 ± 0·01 mg kg-1 ). At relapse, mcPV shifted to mucosal dominant PV (mPV; 40%), pemphigus foliaceus (PF) (20%) or 'other' (20%). In contrast, relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Patients with both anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies at onset had recurrence with anti-Dsg3 antibodies alone (40%), with both anti-Dsg1 and anti-Dsg3 antibodies (30%), with anti-Dsg1 antibody alone (20%) or were subthreshold (10%). CONCLUSIONS: mcPV shows transitions in clinical phenotype and autoantibody profile at relapse. At least 1 mg kg-1 daily of prednisolone, especially for patients with mcPV, and prudent tapering around 0·1 mg kg-1 may lead to better outcomes.
  • Case of autoimmune intraepidermal and subepidermal blistering disease in which autoantibodies to desmoglein 1 and BP230 coexist.
    Sugai T, Ujiie H, Nakamura H, Kikuchi K, Iwata H, Shimizu H
    The Journal of dermatology, 46, 6, e215, e216, Jun. 2019, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal
  • 制御性T細胞の機能不全により類天疱瘡抗原への自己抗体産生が誘導される               
    村松 憲, 氏家 英之, 西江 渉, 泉 健太郎, 伊東 孝政, 葭本 倫大, 夏賀 健, 岩田 浩明, 清水 宏, 小林 一郎
    日本皮膚科学会雑誌, 129, 3, 353, 353, (公社)日本皮膚科学会, Mar. 2019
    Japanese
  • 咽頭粘膜まで広範囲に病変を生じた粘膜型尋常性天疱瘡の1例               
    野原 拓馬, 村松 憲, 眞井 翔子, 宮澤 元, 伊東 孝政, 氏家 英之, 岩田 浩明, 清水 宏, 渡辺 雅子
    日本皮膚科学会雑誌, 129, 3, 355, 355, (公社)日本皮膚科学会, Mar. 2019
    Japanese
  • H.pylori除菌療法後に生じた皮疹の機序解明               
    伊東 孝政, 氏家 英之, 清水 宏, 阿部 理一郎
    日本皮膚科学会雑誌, 129, 3, 394, 394, (公社)日本皮膚科学会, Mar. 2019
    Japanese
  • Characteristics of IgG subclasses and complement deposition in BP230-type bullous pemphigoid
    M. Zheng, H. Ujiie, H. Iwata, K. Muramatsu, N. Yoshimoto, T. Ito, I. Ujiie, S. Shimizu, K. C. Sato-Matsumura, H. Shimizu
    Journal of the European Academy of Dermatology and Venereology, 33, 3, 595, 600, Mar. 2019, [Peer-reviewed], [Corresponding author]
    English, Scientific journal
  • Fc-binding proteins enhance autoantibody-induced BP180 depletion in pemphigoid.
    Iwata H, Kamaguchi M, Ujiie H, Ujiie I, Natsuga K, Nishie W, Shimizu H
    The Journal of pathology, 247, 3, 371, 380, Mar. 2019, [Peer-reviewed], [International Magazine]
    English, Scientific journal, Immunoglobulins (Igs) consist of two antigen-binding regions (Fab) and one constant region (Fc). Protein A and protein G are bacterial proteins used for the purification of IgG by virtue of their high affinities for the Fc fragment. Rheumatoid factors are autoantibodies against IgG Fc fragments, which are present in the body under physiological conditions. Little is known about the influence of Fc-binding proteins on the pathogenicity of antibody-induced autoimmune diseases. Pemphigoid diseases are a group of autoimmune subepidermal blistering disorders that includes bullous pemphigoid and mucous membrane pemphigoid. IgGs targeting the non-collagenous NC16A domain of the 180-kDa bullous pemphigoid antigen (BP180) are known to induce skin fragility in mice and the depletion of BP180 in keratinocytes. In this study, mAb against NC16A in combination with Fc-binding proteins was found to enhance BP180 depletion. Although mAb against the C-terminus of BP180 does not show pathogenicity in vivo or in vitro, mAb treatment with Fc-binding proteins clearly induced skin fragility in mice and BP180 depletion in keratinocytes. Anti-BP180 mAbs and Fc-binding proteins were colocalized in the cytoplasm and at the basement membrane zone. Cell adhesion strengths were decreased in parallel with BP180 amounts. Clinically, bullous pemphigoid patients had higher rheumatoid factor titers than controls. Anti-BP180 mAb in combination with high-titer rheumatoid factor serum was found to enhance BP180 depletion. Furthermore, saliva from mucous membrane pemphigoid patients contained larger quantities of bacteria and Fc-binding proteins than controls. Our results suggest that Fc-binding proteins (rheumatoid factor or protein G) may enhance the pathogenicity of autoantibodies in pemphigoid diseases. Copyright © 2018 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
  • Efficient gene reframing therapy for recessive dystrophic epidermolysis bullosa using CRISPR/Cas9.
    Takashima S, Shinkuma S, Fujita Y, Nomura T, Ujiie H, Natsuga K, Iwata H, Nakamura H, Vorobyev A, Abe R, Shimizu H
    The Journal of investigative dermatology, 139, 8, 1711, 1721, Mar. 2019, [Peer-reviewed], [International Magazine]
    English, Scientific journal, The clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9 system induces site-specific double-strand breaks, which stimulate cellular DNA repair through either the homologous recombination or non-homologous end-joining pathways. The non-homologous end-joining pathway, which is activated more frequently than homologous recombination, is prone to introducing small insertions and/or deletions at the double-strand break site, leading to changes in the reading frame. We hypothesized that the non-homologous end-joining pathway is applicable to genetic diseases caused by a frameshift mutation through restoration of the reading frame. Recessive dystrophic epidermolysis bullosa is a hereditary skin disorder caused by mutations in COL7A1. In this study, we applied gene reframing therapy to a recurrent frameshift mutation, c.5819delC, in COL7A1, which results in a premature termination codon. CRISPR/Cas9 targeting this specific mutation site was delivered to recessive dystrophic epidermolysis bullosa patient fibroblasts. After genotyping a large collection of gene-edited fibroblast clones, we identified a significant number (17/50) of clones in which the frameshift in COL7A1 was restored. The reframed COL7 was functional, as shown by triple-helix formation assay in vitro, and was correctly distributed in the basement membrane zone in mice. Our data suggest that mutation site-specific non-homologous end-joining might be a highly efficient gene therapy for inherited disorders caused by frameshift mutations.
  • The direct binding of collagen XVII and collagen IV is disrupted by pemphigoid autoantibodies.
    Mayumi Kamaguchi, Hiroaki Iwata, Wataru Nishie, Ellen Toyonaga, Hideyuki Ujiie, Ken Natsuga, Yoshimasa Kitagawa, Hiroshi Shimizu
    Laboratory investigation; a journal of technical methods and pathology, 99, 1, 48, 57, Jan. 2019, [Peer-reviewed], [International Magazine]
    English, Scientific journal, The basement membrane zone (BMZ) is framed by hemidesmosomes and extracellular matrix (ECM) including collagen IV (COL4). Hemidesmosomes are multiprotein complexes that include collagen XVII (COL17). BMZ proteins can be targeted in autoimmune subepidermal blistering diseases, e.g., pemphigoid targeting COL17. The blistering mechanisms in pemphigoid have not been fully elucidated, especially in mucous membrane pemphigoid (MMP), which mainly affects the mucosa. In this study, we showed that oral lesions in pemphigoid may be attributed to the inhibition of protein-protein interactions by autoantibodies. Using immunoprecipitation, we revealed that COL17 directly binds to COL4 in normal human keratinocytes and normal human oral keratinocytes. In particular, the C-terminus of COL17 is binding site to COL4 in oral keratinocytes. The precise COL4-binding region on COL17 was determined by protein-protein binding assay to be from amino acid Gly1175 to Asp1340 on the C-terminus. MMP-IgG or mAb recognizing the C-terminus hindered the interaction of COL17 with COL4 in oral keratinocytes. Furthermore, keratinocyte adhesion strength to COL4-coated plates was significantly reduced by the treatment of mAb against the C-terminus. In addition, the inflammatory infiltrates around perilesions were significantly less in MMP compared to BP. These results indicate that pemphigoid IgG targeting the C-terminus plays a pathogenic role in blister formation in the oral mucosa to inhibit protein interactions with less inflammation.
  • Immune Reaction to Type XVII Collagen Induces Intramolecular and Intermolecular Epitope Spreading in Experimental Bullous Pemphigoid Models.
    Hideyuki Ujiie, Norihiro Yoshimoto, Ken Natsuga, Ken Muramatsu, Hiroaki Iwata, Wataru Nishie, Hiroshi Shimizu
    Frontiers in immunology, 10, 1410, 1410, 2019, [Peer-reviewed], [Lead author, Corresponding author], [International Magazine]
    English, Scientific journal, Bullous pemphigoid (BP), the most common autoimmune blistering disease, is induced by autoantibodies to type XVII collagen (COL17). Previous studies demonstrated that COL17 harbors several epitopes targeted by autoreactive T and B cells and that the target epitopes change sequentially during the disease course. To elucidate the details of the humoral immune response to COL17, we used an active BP mouse model in which BP is induced by the adoptive transfer of spleen cells from wild-type mice immunized with human COL17-expressing skin grafting to immunodeficient COL17-humanized (Rag-2-/-, mouse Col17-/-, human COL17+) mice. By immunoblot analysis, antibodies to the NC16A domain and other extracellular domains (ECDs) of COL17 were detected earlier than antibodies to intracellular domains (ICDs) in the active BP model. Time course analysis by enzyme-linked immunosorbent assay demonstrated a delayed peak of antibodies to ICD epitopes in active BP model. The blockade of CD40-CD40 ligand interaction soon after the adoptive transfer suppressed the production of antibodies to the non-collagenous 16A (NC16A) domain but not to an ICD epitope, suggesting the sequential activation from T and B cells against the ECD epitopes including the NC16A domain to those against ICD epitopes in vivo. Both wild-type mice immunized with a fragment of the NC16A domain and the recipients of those spleen cells produced IgG antibodies to ICD and ECD epitopes, showing intramolecular epitope spreading from the NC16A domain to other epitopes of COL17. Furthermore, we found that a portion of the active BP model mice show intermolecular epitope spreading from human COL17 to murine BP230. The appearance of antibodies to ICD epitopes of COL17 or of antibodies to murine BP230 did not correlate with the skin changes in the mice, suggesting that those antibodies have low pathogenicity. These results suggest that the immune response to the ECD epitopes of COL17, especially to the NC16A domain, triggers intramolecular, and intermolecular epitope spreading to ICD epitopes of COL17 and to murine BP230. These novel findings provide insight into the mechanism of epitope spreading in organ-specific, antibody-mediated autoimmune disorders.
  • Autoantibodies undetectable by chemiluminescent enzyme immunoassay require extended antigen-antibody reaction time for detection
    Mai Y, Ujiie H, Higashi T, Yamagami J, Iwata H, Shimizu H
    Br J Dermatol, 180, 1, 215, 216, Jan. 2019, [Peer-reviewed], [Corresponding author]
    English, Scientific journal
  • Regulatory T-cell dysfunction induces autoantibodies to bullous pemphigoid antigens in mice and human subjects.
    Ken Muramatsu, Hideyuki Ujiie, Ichiro Kobayashi, Wataru Nishie, Kentaro Izumi, Takamasa Ito, Norihiro Yoshimoto, Ken Natsuga, Hiroaki Iwata, Hiroshi Shimizu
    The Journal of allergy and clinical immunology, 142, 6, 1818, 1830, Dec. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal, BACKGROUND: Regulatory T (Treg) cells play a crucial role in peripheral immune tolerance in multiple organs, including the skin. Thus far, the effect of peripheral immune tolerance failure on autoantibody-related autoimmune reactions to the skin is unclear. OBJECTIVE: We sought to elucidate the target autoantigens in the skin under the condition of Treg cell dysfunction caused by forkhead box P3 (Foxp3) gene mutations in scurfy mice and patients with immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. METHODS: Sera and skin from scurfy mice and sera from patients with IPEX syndrome were analyzed to detect target autoantigens by using immunofluorescence studies, ELISAs, and immunoblotting. The pathogenicity of scurfy IgG was examined by using a passive transfer experiment. CD4+ T cells from scurfy mice were transferred to immunodeficient mice to examine their pathogenicity. Signal transducer and activator of transcription 6 (Stat6)-/- scurfy mice were analyzed to further clarify the molecular pathway of autoantibody production. Follicular helper T-cell counts are measured in Stat6-/- scurfy mice and scurfy mice. RESULTS: Scurfy mice spontaneously generated IgG autoantibodies to the dermal-epidermal junction, which had been class-switched from IgM within 12 days after birth. The target autoantigens were murine BP230 and type XVII collagen (COL17). The scurfy polyclonal autoantibodies did not induce skin fragility in neonatal mice. Autoantibody production was induced by CD4+ T cells from scurfy mice and was ameliorated by Stat6 gene knockout in association with a decrease of follicular helper T cells. We also identified autoantibodies to COL17 and BP230 in patients with IPEX syndrome and found an association between production of autoantibodies to COL17 and an eczematous skin phenotype. CONCLUSIONS: Dysregulation of Treg cells generates autoantibodies to COL17 and BP230 in vivo.
  • The identification of autoantigens in mucous membrane pemphigoid using immortalized oral mucosal keratinocytes.
    Kamaguchi M, Iwata H, Miyauchi T, Ujiie H, Ujiie I, Nomura T, Ohga N, Shimizu H, Kitagawa Y
    Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology, 48, 1, 60, 67, Sep. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal, BACKGROUND: Mucous membrane pemphigoid (MMP) is a rare chronic autoimmune subepithelial blistering disorder, targeting multiple basement membrane zone (BMZ) proteins including collagen XVII (COL17). Circulating autoantibodies of MMP are often undetected due to their lower titers. The oral mucosa is a valuable substrate for the detection of autoantibodies in MMP patients. However, obtaining normal human oral mucosa is more difficult than obtaining normal human skin. We established immortalized normal human oral mucosal keratinocytes (OMKs) and performed immunoblotting using immortalized OMK lysate for detecting autoantigens in MMP. METHODS: Immortalized OMKs were generated from primary OMKs using E6/E7 proteins of HPV. We compared the protein expression levels of major BMZ proteins between primary OMKs and immortalized OMKs. We performed immunoblotting to detect autoantigens using cell lysates from immortalized OMKs in 30 MMP patients. RESULTS: There were no significant differences between primary OMKs and immortalized OMKs in terms of protein expression levels of the BMZ proteins, including COL17, laminin 332, integrin α6/β4, collagen VII, and collagen IV. Cell lysates of immortalized OMKs effectively identified MMP autoantigens in 60% (18/30) of MMP sera. We found an interesting case of MMP whose autoantibodies preferentially reacted to the 120-kD protein that is an ectodomain of COL17. CONCLUSION: We demonstrated that a cell lysate of immortalized OMKs is a reliable substrate for the detection of MMP autoantigens. This newly developed immunoblotting analysis method promises to contribute to the diagnosis of MMP.
  • Detection of anti-BP180 NC16A autoantibodies after the onset of dipeptidyl peptidase-IV inhibitor-associated bullous pemphigoid: a report of three patients.
    Mai Y, Nishie W, Izumi K, Yoshimoto N, Morita Y, Watanabe M, Toyonaga E, Ujiie H, Iwata H, Fujita Y, Nomura T, Sato-Matsumura KC, Shimizu S, Shimizu H
    The British journal of dermatology, 179, 3, 790, 791, Sep. 2018, [Peer-reviewed]
  • Potential role of extracellular vesicle-mediated antigen presentation in Helicobacter pylori hypersensitivity during eradication therapy.
    Takamasa Ito, Takashi Shiromizu, Shunsuke Ohnishi, Shotaro Suzuki, Katsuhiro Mabe, Akito Hasegawa, Hideyuki Ujiie, Yasuyuki Fujita, Yuichi Sato, Shuji Terai, Mototsugu Kato, Masahiro Asaka, Takeshi Tomonaga, Hiroshi Shimizu, Riichiro Abe
    The Journal of allergy and clinical immunology, 142, 2, 672, 676, Aug. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Mucosal substrates successfully identify the autoantigen in a case of mucous membrane pemphigoid.
    Kamaguchi M, Iwata H, Ujiie H, Ohga N, Kitagawa Y, Shimizu H
    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 16, 8, 1032, 1034, Aug. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Schleimhautsubstrate identifizieren erfolgreich das Autoantigen bei einem Schleimhautpemphigoid.
    Mayumi Kamaguchi, Hiroaki Iwata, Hideyuki Ujiie, Noritaka Ohga, Yoshimasa Kitagawa, Hiroshi Shimizu
    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 16, 8, 1032, 1035, Aug. 2018, [International Magazine]
    English
  • High Expression of Collagen XVII Compensates for its Depletion Induced by Pemphigoid IgG in the Oral Mucosa.
    Kamaguchi M, Iwata H, Ujiie H, Natsuga K, Nishie W, Kitagawa Y, Shimizu H
    The Journal of investigative dermatology, 138, 8, 1707, 1715, Aug. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal, The basement membrane zone consists of multiple components, including collagen XVII (COL17), which is the target of bullous pemphigoid. To our knowledge, no research has addressed the differences in basement membrane zone components between the skin and oral mucosa; therefore, we investigated the basement membrane zone proteins, with a focus on COL17. The mRNA and protein expression levels of COL17 were significantly higher in oral keratinocytes than in skin keratinocytes. Hemidesmosomal COL17 expression was markedly higher in oral keratinocytes than in skin keratinocytes, and its level was associated with adhesion strength. Oral keratinocytes adhered to the extracellular matrix more tightly than did skin keratinocytes in vitro. Based on these results, we attempt to explain the clinical diversity of bullous pemphigoid. COL17 depletion was more prominent in skin keratinocytes than in oral keratinocytes after treatment with COL17-NC16A mAbs, which have in vivo pathogenicity. COL17 C-terminus mAbs, which are not pathogenic, facilitated COL17 depletion in combination treatment with COL17-NC16A mAbs in both types of keratinocytes. In summary, the greater amount of COL17 in oral keratinocytes than in skin keratinocytes is associated with the higher strength of oral keratinocyte hemidesmosomal adhesion at the basement membrane zone. Our results may explain why bullous pemphigoid blistering tends to be more prevalent in the skin than in the oral mucosa.
  • HLA-DQB1*03:01は非炎症型DPP-4阻害薬関連水疱性類天疱瘡のバイオマーカーである               
    氏家 英之, 村松 憲, 岩田 浩明, 西村 真智子, 伊東 孝政, 泉 健太郎, 西江 渉, 清水 宏, 三好 秀明, 莚田 泰誠, 大関 健志
    日本皮膚科学会雑誌, 128, 8, 1663, 1663, (公社)日本皮膚科学会, Jul. 2018
    Japanese
  • A case of red lunulae after haematopoietic stem cell transplantation.
    Yosuke Mai, Hideyuki Ujiie, Akihiro Iguchi, Hiroshi Shimizu
    European journal of dermatology : EJD, 28, 3, 407, 409, 01 Jun. 2018, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal
  • Sweet's Syndrome Mimicking Anti-Neutrophil Cytoplasmic Antibodies-Associated Vasculitis.
    Hideyuki Kosumi, Mika Watanabe, Ken Natsuga, Toshinari Miyauchi, Chihiro Shiiya, Hideyuki Ujiie, Hiroshi Shimizu
    The American journal of medicine, 131, 6, e241-e242, E242, Jun. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Intravenous IgG Reduces Pathogenic Autoantibodies, Serum IL-6 Levels, and Disease Severity in Experimental Bullous Pemphigoid Models.
    Tetsumasa Sasaoka, Hideyuki Ujiie, Wataru Nishie, Hiroaki Iwata, Makoto Ishikawa, Hiroshi Higashino, Ken Natsuga, Satoru Shinkuma, Hiroshi Shimizu
    The Journal of investigative dermatology, 138, 6, 1260, 1267, Jun. 2018, [Peer-reviewed], [Lead author, Last author], [International Magazine]
    English, Scientific journal
  • Case of anti-p200 pemphigoid accompanying uterine malignancy.
    Hayashi M, Okamura K, Ujiie H, Iwata H, Suzuki T
    The Journal of dermatology, 45, 12, e341-e342, May 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • 落葉状天疱瘡合併の悪性黒色腫に免疫チェックポイント阻害薬を投与した1例               
    前田 拓哉, 柳 輝希, 今福 恵輔, 北村 真也, 秦 洋郎, 泉 健太郎, 氏家 英之, 岩田 浩明, 清水 宏
    日本皮膚科学会雑誌, 128, 5, 1232, 1232, (公社)日本皮膚科学会, May 2018
    Japanese
  • HLA-DQB1*03:01 as a Biomarker for Genetic Susceptibility to Bullous Pemphigoid Induced by DPP-4 Inhibitors.
    Hideyuki Ujiie, Ken Muramatsu, Taisei Mushiroda, Takeshi Ozeki, Hideaki Miyoshi, Hiroaki Iwata, Akinobu Nakamura, Hiroshi Nomoto, Kyu Yong Cho, Norihiro Sato, Machiko Nishimura, Takamasa Ito, Kentaro Izumi, Wataru Nishie, Hiroshi Shimizu
    The Journal of investigative dermatology, 138, 5, 1201, 1204, May 2018, [Peer-reviewed], [Lead author, Corresponding author], [International Magazine]
    English, Scientific journal
  • Maculopapular drug eruption induced by linagliptin.
    Zheng M, Yoshimoto N, Muramatsu K, Ito T, Ujiie H, Shimizu H
    The Australasian journal of dermatology, 59, 4, e287-e288, Apr. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Regulatory T cells in autoimmune skin diseases.
    Ujiie H
    Experimental dermatology, 28, 6, 642, 646, Mar. 2018, [Peer-reviewed], [Invited], [Lead author, Last author, Corresponding author], [International Magazine]
    English, Scientific journal, CD4+ Foxp3+ regulatory T cells (Tregs) are suppressors of immune activation and play a crucial role in the maintenance of peripheral tolerance. Mutations of Foxp3 result in fatal autoimmunity in multiple organs, including the skin, in both humans and mice. Many studies have demonstrated the altered frequency and functions of Tregs, changes in cytokine and chemokine levels related to Tregs and the differences in genetic background regarding Tregs in autoimmune skin disorders. Recent studies have extended our knowledge of certain properties of Tregs, especially skin-resident Tregs. In addition, some novel therapies have been performed by modulating the number and the function of Tregs. This review focuses on the role of Tregs in some autoimmune skin disorders, including alopecia areata, vitiligo, pemphigoid and pemphigus, and systemic sclerosis, and discusses questions that remain to be addressed.
  • Six-month history of a split thumbnail: A quiz
    Yosuke Mai, Hideyuki Ujiie, Takashi Anan, Hajime Miyazawa, Keisuke Imafuku, Kokichi Hamasaka, Hiroshi Shimizu
    Acta Dermato-Venereologica, 98, 2, 297, 298, Medical Journals/Acta D-V, 01 Feb. 2018, [Peer-reviewed], [Corresponding author], [International Magazine]
    English, Scientific journal
  • Diagnosis and Management of Pemphigus: recommendations by an International Panel of Experts.
    Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, Sinha AA, Payne AS, Daneshpazhooh M, Eming R, Jonkman MF, Mimouni D, Borradori L, Kim SC, Yamagami J, Lehman JS, Saleh MA, Culton DA, Czernik A, Zone JJ, Fivenson D, Ujiie H, Wozniak K, Akman-Karakaş A, Bernard P, Korman NJ, Caux F, Drenovska K, Prost-Squarcioni C, Vassileva S, Feldman RJ, Cardones AR, Bauer J, Ioannides D, Jedlickova H, Palisson F, Patsatsi A, Uzun S, Yayli S, Zillikens D, Amagai M, Hertl M, Schmidt E, Aoki V, Grando SA, Shimizu H, Baum S, Cianchini G, Feliciani C, Iranzo P, Mascaró JM Jr, Kowalewski C, Hall R, Groves R, Harman KE, Marinkovich MP, Maverakis E, Werth VP
    Journal of the American Academy of Dermatology, 82, 3, 575, 585, Feb. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal, BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.
  • 落葉状天疱瘡合併悪性黒色腫に免疫チェックポイント阻害薬を投与した1例               
    前田 拓哉, 柳 輝希, 今福 恵輔, 北村 真也, 秦 洋郎, 泉 健太郎, 氏家 英之, 岩田 浩明, 清水 宏
    日本皮膚科学会雑誌, 128, 1, 61, 61, (公社)日本皮膚科学会, Jan. 2018
    Japanese
  • 爪線状苔癬の1例               
    眞井 洋輔, 氏家 英之, 宮澤 元, 今福 恵輔, 清水 宏, 阿南 隆, 浜坂 幸吉
    日本皮膚科学会雑誌, 128, 1, 64, 64, (公社)日本皮膚科学会, Jan. 2018
    Japanese
  • 口腔粘膜基質を用いて抗原同定したBP180型粘膜類天疱瘡の1例               
    鎌口 真由美, 岩田 浩明, 氏家 英之, 清水 宏, 大賀 則孝, 北川 善政
    日本皮膚科学会雑誌, 128, 1, 64, 64, (公社)日本皮膚科学会, Jan. 2018
    Japanese
  • Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid.
    Mayumi Kamaguchi, Hiroaki Iwata, Inkin Ujiie, Hideyuki Ujiie, Jun Sato, Yoshimasa Kitagawa, Hiroshi Shimizu
    Frontiers in medicine, 5, 20, 20, 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal, Mucous membrane pemphigoid (MMP) is a rare organ-specific autoimmune subepithelial blistering disease with predominantly mucosal erosions, most frequently affecting the gingiva. Erosions in the oral cavity usually result in markedly decreased quality of life. The major autoantigens are BP180 and laminin332, which are components of basement membrane proteins in the skin and mucosa. Diagnosis is usually difficult due to histological destruction of the tissue and low autoantibody titers. In this study, we evaluated the diagnostic value of direct immunofluorescence (DIF) using non-lesional buccal mucosa in seven cases of MMP. In all seven patients, gingival lesions were clinically observed, and in one of the seven patients, buccal lesions were also clinically observed. First, we performed DIF to detect tissue-bound autoantibodies and complement. DIF from non-lesional buccal mucosa revealed linear deposits of IgG and C3 at the basement membrane zone in all cases. To detect autoantibodies, indirect immunofluorescence (IIF), BP180-NC16A ELISA and immunoblotting were performed. Surprisingly, circulating autoantibodies were unable to be detected in any of the cases by ELISA, IIF, or immunoblotting. Furthermore, histological separation was observed in one patient. In conclusion, DIF using non-lesional buccal mucosa was found to be superior to histological and serological tests for diagnosing mucous membrane pemphigoid. The procedure is technically easy and has high diagnostic value.
  • Appearance of antidesmocollin 1 autoantibodies leading to a vegetative lesion in a patient with pemphigus vulgaris.
    Yamaguchi Y, Shinkuma S, Ishii N, Takashima S, Natsuga K, Ujiie H, Iwata H, Nomura T, Fujita Y, Hamasaka A, Hamasaka K, Hashimoto T, Shimizu H
    The British journal of dermatology, 178, 1, 294, 295, Jan. 2018, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Autoantibodies of non-inflammatory bullous pemphigoid hardly deplete type XVII collagen of keratinocytes
    Keisuke Imafuku, Hiroaki Iwata, Mayumi Kamaguchi, Kentaro Izumi, Ken Natsuga, Hideyuki Ujiie, Wataru Nishie, Hiroshi Shimizu
    Experimental Dermatology, 26, 12, 1171, 1174, Blackwell Publishing Ltd, 01 Dec. 2017, [Peer-reviewed], [International Magazine]
    English
  • Complement-independent blistering mechanisms in bullous pemphigoid
    Hiroaki Iwata, Hideyuki Ujiie
    Experimental Dermatology, 26, 12, 1235, 1239, Blackwell Publishing Ltd, 01 Dec. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Using immune checkpoint inhibitors without exacerbation in a melanoma patient with pemphigus foliaceus
    Takuya Maeda, Teruki Yanagi, Keisuke Imafuku, Shinya Kitamura, Hiroo Hata, Kentaro Izumi, Hideyuki Ujiie, Hiroaki Iwata, Hiroshi Shimizu
    INTERNATIONAL JOURNAL OF DERMATOLOGY, 56, 12, 1477, 1479, Dec. 2017, [Peer-reviewed], [International Magazine]
    English
  • C-Terminal Processing of Collagen XVII Induces Neoepitopes for Linear IgA Dermatosis Autoantibodies
    Ellen Toyonaga, Wataru Nishie, Kentaro Izumi, Ken Natsuga, Hideyuki Ujiie, Hiroaki Iwata, Jun Yamagami, Yoshiaki Hirako, Daisuke Sawamura, Wataru Fujimoto, Hiroshi Shimizu
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 12, 2552, 2559, Dec. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Anti-idiotypic Antibodies against BP-IgG Prevent Type XVII Collagen Depletion
    Mayumi Kamaguchi, Hiroaki Iwata, Yuiko Mori, Ellen Toyonaga, Hideyuki Ujiie, Yoshimasa Kitagawa, Hiroshi Shimizu
    FRONTIERS IN IMMUNOLOGY, 8, 1669, 1669, Nov. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Detection of mucous membrane pemphigoid autoantibodies by full-length BP180 enzyme-linked immunosorbent assay
    Kentaro Izumi, Wataru Nishie, Yosuke Mai, Hideyuki Ujiie, Hiroaki Iwata, Ken Natsuga, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGICAL SCIENCE, 88, 2, 247, 248, Nov. 2017, [Peer-reviewed], [International Magazine]
    English
  • Bullous pemphigoid following the replacement of vildagliptin with anagliptin.
    Kazumasa Oya, Masanao Fujii, Shijima Taguchi, Wataru Nishie, Kentaro Izumi, Hiroshi Shimizu
    The Journal of dermatology, 44, 10, e238-e239, Oct. 2017, [International Magazine]
    English
  • Chemiluminescent enzyme immunoassay failed to detect anti-desmoglein 3 antibodies in a case of pemphigus vulgaris
    Yosuke Mai, Hideyuki Ujiie, Machiko Nishimura, Hiroshi Koga, Yuka Maya, Keiko Shiba-Tokuchi, Yasuyuki Fujita, Hiroaki Iwata, Yohei Mikawa, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGY, 44, 10, E242, E243, Oct. 2017, [Peer-reviewed], [International Magazine]
    English
  • Oral mucosa is a useful substrate for detecting autoantibodies of mucous membrane pemphigoid.
    Kamaguchi M, Iwata H, Ujiie H, Izumi K, Natsuga K, Nishie W, Asaka T, Kitagawa Y, Shimizu H
    The British journal of dermatology, 178, 2, e119, e121, Sep. 2017, [Peer-reviewed]
  • Generalized Pustular Psoriasis
    Hideyuki Kosumi, Takamasa Ito, Yasuyuki Fujita, Kentaro Izumi, Yuka Maya, Teruki Yanagi, Ken Natsuga, Hideyuki Ujiie, Satoru Shinkuma, Toshifumi Nomura, Naoya Sadanobu, Hiroshi Shimizu
    JOURNAL OF PEDIATRICS, 188, 305, +, Sep. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • 複数の自己抗体を検出した自己免疫性表皮下水疱症の1例               
    澤田 匡秀, 肥田 時征, 氏家 英之, 泉 健太郎, 西江 渉, 宇原 久
    日本皮膚科学会雑誌, 127, 9, 2117, 2117, (公社)日本皮膚科学会, Aug. 2017
    Japanese
  • Postherpetic abdominal pseudohernia
    Ken Muramatsu, Hideyuki Ujiie, Hiroshi Shimizu
    BMJ-BRITISH MEDICAL JOURNAL, 358, Jul. 2017, [Peer-reviewed]
    English
  • Late-onset skin involvement on the forehead in multicentric Castleman disease
    Kazumasa Sato, Satoru Shinkuma, Hideyuki Ujiie, Toshifumi Nomura, Yasuyuki Fujita, Riichiro Abe, Hiroshi Shimizu, Katsuya Fujimoto, Kanako C. Hatanaka, Yoshihiro Matsuno
    INTERNATIONAL JOURNAL OF DERMATOLOGY, 56, 7, E152, E153, Jul. 2017, [Peer-reviewed], [International Magazine]
    English
  • Type XVII collagen coordinates proliferation in the interfollicular epidermis
    Mika Watanabe, Ken Natsuga, Wataru Nishie, Yasuaki Kobayashi, Giacomo Donati, Shotaro Suzuki, Yu Fujimura, Tadasuke Tsukiyama, Hideyuki Ujiie, Satoru Shinkuma, Hideki Nakamura, Masamoto Murakami, Michitaka Ozaki, Masaharu Nagayama, Fiona M. Watt, Hiroshi Shimizu
    ELIFE, 6, Jul. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • A case of bullous pemphigoid presenting with severe oedema of the hands during recurrence
    Shinichi Nakazato, Hideyuki Ujiie, Yuka Inamura, Machiko Nishimura, Hiroaki Iwata, Hiroshi Shimizu
    EUROPEAN JOURNAL OF DERMATOLOGY, 27, 4, 421, 422, Jul. 2017, [Peer-reviewed], [International Magazine]
    English
  • Postherpetic abdominal pseudohernia.
    Muramatsu K, Ujiie H, Shimizu H
    BMJ (Clinical research ed.), 358, j2786, Jul. 2017, [Peer-reviewed]
  • Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016
    Enno Schmidt, Volker Spindler, Ruediger Eming, Masayuki Amagai, Frank Antonicelli, John F. Baines, Meriem Belheouane, Philippe Bernard, Luca Borradori, Marzia Caproni, Giovanni Di Zenzo, Sergei Grando, Karen Harman, Marcel F. Jonkman, Hiroshi Koga, Ralf J. Ludwig, Andrew P. Kowalczyk, Eliane J. Mueller, Wataru Nishie, Hendri Pas, Aimee S. Payne, Christian D. Sadik, Allan Seppanen, Jane Setterfield, Hiroshi Shimizu, Animesh A. Sinha, Eli Sprecher, Michael Sticherling, Hideyuki Ujiie, Detlef Zillikens, Michael Hertl, Jens Waschke
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 6, 1199, 1203, Jun. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Linear IgA Bullous Dermatosis Associated with Immunoglobulin Light-chain Amyloidosis
    Yasuyuki Yamaguchi, Hideyuki Ujiie, Hiroyuki Ohigashi, Hiroaki Iwata, Ken Muramatsu, Tomoyuki Endou, Takanori Teshima, Hiroshi Shimizu
    ACTA DERMATO-VENEREOLOGICA, 97, 4, 528, 529, Apr. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Hypertrophic lupus erythematosus successfully treated with hydroxychloroquine
    Norihiro Yoshimoto, Satoru Shinkuma, Hideyuki Ujiie, Toshifumi Nomura, Yasuyuki Fujita, Riichiro Abe, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGY, 44, 3, E48, E49, Mar. 2017, [Peer-reviewed], [International Magazine]
    English
  • Linear IgA Bullous Dermatosis in a Pregnant Woman with Autoantibodies to the Non-collagenous 16A Domain of Type XVII Collagen
    Kenshi Matsuura, Hideyuki Ujiie, Masahiro Hayashi, Ken Muramatsu, Junko Yoshizawa, Takamasa Ito, Hiroaki Iwata, Tamio Suzuki, Hiroshi Shimizu
    ACTA DERMATO-VENEREOLOGICA, 97, 3, 404, 405, Mar. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Lichenoid drug eruption caused by clonazepam
    K. Muramatsu, H. Ujiie, K. Natsuga, W. Nishie, H. Shimizu
    JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY, 31, 2, E117, E118, Feb. 2017, [Peer-reviewed]
    English
  • Recurrence of juvenile dermatomyositis 8 years after remission
    Ken Muramatsu, Hideyuki Ujiie, Mayumi Yokozeki, Ichiro Tsukinaga, Mai Ito, Takaaki Shikano, Akira Suzuki, Yusuke Tozawa, Ichiro Kobayashi
    JAAD Case Reports, 3, 1, 29, 32, Elsevier Inc, 01 Jan. 2017, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Image Gallery: Vasculo-Behçet disease.
    Muramatsu K, Ujiie H, Ito T, Fujita Y, Inokuma D, Tsukinaga I, Abe T, Shirai S, Fukuda N, Shimizu H
    The British journal of dermatology, 175, 6, e150, Dec. 2016, [Peer-reviewed]
  • Macropinocytosis of type XVII collagen induced by bullous pemphigoid IgG is regulated via protein kinase C
    Hiroaki Iwata, Mayumi Kamaguchi, Hideyuki Ujiie, Machiko Nishimura, Kentaro Izumi, Ken Natsuga, Satoru Shinkuma, Wataru Nishie, Hiroshi Shimizu
    LABORATORY INVESTIGATION, 96, 12, 1301, 1310, Dec. 2016, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Autoantibody Profile Differentiates between Inflammatory and Noninflammatory Bullous Pemphigoid
    Kentaro Izumi, Wataru Nishie, Yosuke Mai, Mayumi Wada, Ken Natsuga, Hideyuki Ujiie, Hiroaki Iwata, Jun Yamagami, Hiroshi Shimizu
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 11, 2201, 2210, Nov. 2016, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Bullous pemphigoid suggestive of complement-independent blister formation with anti-BP180 IgG4 autoantibodies.
    Dainichi T, Nishie W, Yamagami Y, Sonobe H, Ujiie H, Kaku Y, Kabashima K
    The British journal of dermatology, 175, 1, 187, 190, Jul. 2016, [Peer-reviewed]
  • Cutaneous-type pemphigus vulgaris successfully treated with topical corticosteroids
    Hideyuki Ujiie, Satoru Aoyagi, Keita Horie, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGY, 43, 7, 840, 841, Jul. 2016, [Peer-reviewed], [International Magazine]
    English
  • Epitope-Dependent Pathogenicity of Antibodies Targeting a Major Bullous Pemphigoid Autoantigen Collagen XVII/BP180
    Mayumi Wada, Wataru Nishie, Hideyuki Ujiie, Kentaro Izumi, Hiroaki Iwata, Ken Natsuga, Hideki Nakamura, Yoshimasa Kitagawa, Hiroshi Shimizu
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 5, 938, 946, May 2016, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • gamma delta T Cells Protect the Liver and Lungs of Mice from Autoimmunity Induced by Scurfy Lymphocytes
    Hideyuki Ujiie, Ethan M. Shevach
    JOURNAL OF IMMUNOLOGY, 196, 4, 1517, 1528, Feb. 2016, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Anti-BP180-type mucous membrane pemphigoid: report of two cases
    Mayumi Wada, Jun Sato, Masanobu Shindoh, Hideyuki Ujiie, Ken Natsuga, Wataru Nishie, Hiroshi Shimizu, Yoshimasa Kitagawa
    ODONTOLOGY, 104, 1, 114, 118, Jan. 2016, [Peer-reviewed], [Domestic magazines]
    English, Scientific journal
  • Kimura disease associated with severe visual dysfunction due to remarkable periorbital involvement
    Mika Watanabe, Hideyuki Ujiie, Nao Saito, Riichiro Abe, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGY, 42, 9, 924, 925, Sep. 2015, [Peer-reviewed], [International Magazine]
    English
  • Autoantibodies to Multiple Epitopes on the Non-Collagenous-1 Domain of Type VII Collagen Induce Blisters
    Artem Vorobyev, Hideyuki Ujiie, Andreas Recke, Jacqueline J. A. Buijsrogge, Marcel F. Jonkman, Hendri H. Pas, Hiroaki Iwata, Takashi Hashimoto, Soo-Chan Kim, Jong Hoon Kim, Richard Groves, Unni Samavedam, Yask Gupta, Enno Schmidt, Detlef Zillikens, Hiroshi Shimizu, Ralf J. Ludwig
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 135, 6, 1565, 1573, Jun. 2015, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Context-Dependent Regulation of Collagen XVII Ectodomain Shedding in Skin
    Wataru Nishie, Ken Natsuga, Hiroaki Iwata, Kentaro Izumi, Hideyuki Ujiie, Ellen Toyonaga, Hiroo Hata, Hideki Nakamura, Hiroshi Shimizu
    AMERICAN JOURNAL OF PATHOLOGY, 185, 5, 1361, 1371, May 2015, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • In vivo analysis of IgE autoantibodies in bullous pemphigoid: A study of 100 cases
    Reine Moriuchi, Wataru Nishie, Hideyuki Ujiie, Ken Natsuga, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGICAL SCIENCE, 78, 1, 21, 25, Apr. 2015, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • IgE autoantibodies in bullous pemphigoid: Supporting role, or leading player?
    Hideyuki Ujiie
    JOURNAL OF DERMATOLOGICAL SCIENCE, 78, 1, 5, 10, Apr. 2015, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Bullous Pemphigoid Autoantibodies Directly Induce Blister Formation without Complement Activation
    Hideyuki Ujiie, Tetsumasa Sasaoka, Kentaro Izumi, Wataru Nishie, Satoru Shinkuma, Ken Natsuga, Hideki Nakamura, Akihiko Shibaki, Hiroshi Shimizu
    JOURNAL OF IMMUNOLOGY, 193, 9, 4415, 4428, Nov. 2014, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Paper-Based ELISA for the Detection of Autoimmune Antibodies in Body Fluid-The Case of Bullous Pemphigoid
    Chao-Kai Hsu, Hsin-Yu Huang, Wan-Rung Chen, Wataru Nishie, Hideyuki Ujiie, Ken Natsuga, Shu-Ting Fan, Hsi-Kai Wang, Julia Yu-Yun Lee, Wei-Lun Tsai, Hiroshi Shimizu, Chao-Min Cheng
    ANALYTICAL CHEMISTRY, 86, 9, 4605, 4610, May 2014, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Novel ADAR1 mutations including a single amino acid deletion in the deaminase domain underlie dyschromatosis symmetrica hereditaria in Japanese families
    Michihiro Kono, Mutsumi Suganuma, Masashi Akiyama, Yasutomo Ito, Hideyuki Ujiie, Kenichi Morimoto
    INTERNATIONAL JOURNAL OF DERMATOLOGY, 53, 3, E194, E196, Mar. 2014, [Peer-reviewed], [International Magazine]
    English
  • Tubular Spitz Naevus Mimicking Eccrine Spiradenoma
    Yasuyuki Fujita, Satoru Aoyagi, Masumi Tsujiwaki, Erina Homma, Hideyuki Ujiie, Hiromi Fujita, Kanako C. Hatanaka, Hiroshi Shimizu
    ACTA DERMATO-VENEREOLOGICA, 94, 1, 114, 115, 2014, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Linear Congenital Molluscum Contagiosum on the Coccygeal Region
    Hideyuki Ujiie, Satoru Aoyagi, Yu Hirata, Rinko Osawa, Hiroshi Shimizu
    PEDIATRIC DERMATOLOGY, 30, 5, E83, E84, Sep. 2013, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Coexistence case of bullous pemphigoid and pemphigus foliaceus
    Masumi Tsujiwaki, Riichiro Abe, Yukiko Nomura, Machiko Nishimura, Daichi Hoshina, Satoru Shinkuma, Ken Natsuga, Hideyuki Ujiie, Ken Arita, Hiroshi Shimizu
    EUROPEAN JOURNAL OF DERMATOLOGY, 23, 4, 552, 553, Jul. 2013, [Peer-reviewed], [International Magazine]
    English
  • Confluent and reticulated papillomatosis associated with 15q tetrasomy syndrome
    Hanako Koguchi, Hideyuki Ujiie, Satoru Aoyagi, Rinko Osawa, Hiroshi Shimizu
    Acta Dermato-Venereologica, 93, 2, 202, 203, 2, Mar. 2013, [Peer-reviewed], [International Magazine]
    English
  • A Novel Keratin 5 Mutation in an African Family with Epidermolysis Bullosa Simplex Indicates the Importance of the Amino Acid Located at the Boundary Site Between the H1 and Coil 1A Domains
    Satoru Shinkuma, Wataru Nishie, Witold K. Jacyk, Ken Natsuga, Hideyuki Ujiie, Hideki Nakamura, Masashi Akiyama, Hiroshi Shimizu
    ACTA DERMATO-VENEREOLOGICA, 93, 5, 585, 587, 2013, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Efficacy of N-(3,4-dimethoxycinnamoyl)-anthranilic acid (tranilast) against eruptive syringoma: Report of two cases and review of published work
    Keita Horie, Satoru Shinkuma, Yasuyuki Fujita, Hideyuki Ujiie, Satoru Aoyagi, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGY, 39, 12, 1044, 1046, Dec. 2012, [Peer-reviewed], [International Magazine]
    English
  • Evidence for pathogenicity of autoreactive T cells in autoimmune bullous diseases shown by animal disease models
    Hideyuki Ujiie, Hiroshi Shimizu
    EXPERIMENTAL DERMATOLOGY, 21, 12, 901, 905, Dec. 2012, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Coexistence of pustular and vegetative pyoderma gangrenosum in a patient with myelodysplastic syndrome
    Wakana Omiya, Hideyuki Ujiie, Masashi Akiyama, Kentaro Izumi, Akio Shigematsu, Masahiro Onozawa, Hiroshi Shimizu
    EUROPEAN JOURNAL OF DERMATOLOGY, 22, 5, 711, 712, Sep. 2012, [Peer-reviewed], [International Magazine]
    English
  • The beta 9 Loop Domain of PA-PLA(1)alpha Has a Crucial Role in Autosomal Recessive Woolly Hair/Hypotrichosis
    Satoru Shinkuma, Asuka Inoue, Junken Aoki, Wataru Nishie, Ken Natsuga, Hideyuki Ujiie, Toshifumi Nomura, Riichiro Abe, Masashi Akiyama, Hiroshi Shimizu
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 132, 8, 2093, 2095, Aug. 2012, [Peer-reviewed], [International Magazine]
    English
  • Antibodies to Pathogenic Epitopes or Type XVII Collagen Cause Skin Fragility in a Complement-Dependent and -Independent Manner
    Ken Natsuga, Wataru Nishie, Satoru Shinkuma, Hideyuki Ujiie, Machiko Nishimura, Daisuke Sawamura, Hiroshi Shimizu
    JOURNAL OF IMMUNOLOGY, 188, 11, 5792, 5799, Jun. 2012, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Pathogenesis of Bullous Pemphigoid
    Hideyuki Ujiie, Wataru Nishie, Hiroshi Shimizu
    IMMUNOLOGY AND ALLERGY CLINICS OF NORTH AMERICA, 32, 2, 207, +, May 2012, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Mucous membrane pemphigoid with generalized blisters: IgA and IgG autoantibodies target both laminin-332 and type XVII collagen.
    Hayashi I, Shinkuma S, Shimizu S, Natsuga K, Ujiie H, Yasui C, Tsuchiya K, Nishie W, Shimizu H
    The British journal of dermatology, 166, 5, 1116, 1120, 5, May 2012, [Peer-reviewed], [International Magazine]
    English, Scientific journal, Mucous membrane pemphigoid (MMP) is a mucous membrane-dominated, subepidermal autoimmune blistering disease in which autoantibodies usually react with the C-terminal domain of type XVII collagen (COL17) or with laminin-332. Only a few cases of MMP with widespread blisters have been reported. Serologically, IgA and IgG class autoantibodies directed against COL17 or IgG autoantibodies directed against laminin-332 in patients with MMP have been well documented. MMP cases in which IgA reacts with laminin-332, however, are extremely rare. We report a case of MMP in a 67-year-old man. Clinical examination revealed extensive mucosal lesions as well as generalized blisters and erosions that healed with scar formation. The disease was intractable to treatment with systemic steroids. Interestingly, in addition to IgG directed against laminin-332 and the noncollagenous 16A (NC16A) and C-terminal domains of COL17, circulating IgA reacting with laminin-332 and with the NC16A domain of COL17 was also detected. This is the first MMP case with circulating IgA and IgG autoantibodies against both laminin-332 and COL17.
  • Intraepidermal neutrophilic IgA pemphigus successfully treated with dapsone
    Yu Hirata, Riichiro Abe, Kazuhiro Kikuchi, Asuka Hamasaka, Satoru Shinkuma, Toshifumi Nomura, Wataru Nishie, Ken Arita, Hiroshi Shimizu
    EUROPEAN JOURNAL OF DERMATOLOGY, 22, 2, 282, 283, Mar. 2012, [Peer-reviewed], [International Magazine]
    English
  • Noncollagenous 16A domain of type XVII collagen-reactive CD4(+) T cells play a pivotal role in the development of active disease in experimental bullous pemphigoid model
    Hideyuki Ujiie, Akihiko Shibaki, Wataru Nishie, Satoru Shinkuma, Reine Moriuchi, Hongjiang Qiao, Hiroshi Shimizu
    CLINICAL IMMUNOLOGY, 142, 2, 167, 175, Feb. 2012, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Psoriatic onycho-pachydermo-periostitis progressing to generalized pustular psoriasis.
    Watanabe M, Ujiie H, Iitani MM, Abe R, Shimizu H
    Clin Exp Dermatol, 37, 6, 683, 685, 2012, [Peer-reviewed]
  • 塊状のIgA沈着を呈したDuhring疱疹状皮膚炎の1例
    LIN Hsinyu, 氏家英之, 渡邉美佳, 馬場慶子, 阿部理一郎, 清水宏
    臨床皮膚科, 65, 12, 946, 949, (株)医学書院, 01 Nov. 2011
    Japanese
  • Pathogenesis of Bullous Pemphigoid
    Hideyuki Ujiie, Wataru Nishie, Hiroshi Shimizu
    DERMATOLOGIC CLINICS, 29, 3, 439, +, Jul. 2011, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Extremely severe palmoplantar hyperkeratosis in a generalized epidermolytic hyperkeratosis patient with a keratin 1 gene mutation
    Rinko Osawa, Masashi Akiyama, Kentaro Izumi, Hideyuki Ujiie, Kaori Sakai, Ikue Nemoto-Hasebe, Teruki Yanagi, Hiroko Koizumi, Hiroshi Shimizu
    JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 64, 5, 991, 993, May 2011, [Peer-reviewed], [International Magazine]
    English
  • Human IgG1 Monoclonal Antibody against Human Collagen 17 Noncollagenous 16A Domain Induces Blisters via Complement Activation in Experimental Bullous Pemphigoid Model
    Qiang Li, Hideyuki Ujiie, Akihiko Shibaki, Gang Wang, Reine Moriuchi, Hong-jiang Qiao, Hiroshi Morioka, Satoru Shinkuma, Ken Natsuga, Heather A. Long, Wataru Nishie, Hiroshi Shimizu
    JOURNAL OF IMMUNOLOGY, 185, 12, 7746, 7755, Dec. 2010, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Clearance of a Thick Invasive Squamous Cell Carcinoma After Multiple Treatments with Topical Photodynamic Therapy
    Qiang Li, Tianwen Gao, Heather Ann Long, Hideyuki Ujiie
    PHOTOMEDICINE AND LASER SURGERY, 28, 5, 703, 706, Oct. 2010, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Prevalent LIPH Founder Mutations Lead to Loss of P2Y5 Activation Ability of PA-PLA(1)alpha in Autosomal Recessive Hypotrichosis
    Satoru Shinkuma, Masashi Akiyama, Asuka Inoue, Junken Aoki, Ken Natsuga, Toshifumi Nomura, Ken Arita, Riichiro Abe, Kei Ito, Hideki Nakamura, Hideyuki Ujiie, Akihiko Shibaki, Hiraku Suga, Yuichiro Tsunemi, Wataru Nishie, Hiroshi Shimizu
    HUMAN MUTATION, 31, 5, 602, 610, May 2010, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • What's new in bullous pemphigoid
    Hideyuki Ujiie, Akihiko Shibaki, Wataru Nishie, Hiroshi Shimizu
    JOURNAL OF DERMATOLOGY, 37, 3, 194, 204, Mar. 2010, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Blockade of Autoantibody-Initiated Tissue Damage by Using Recombinant Fab Antibody Fragments against Pathogenic Autoantigen
    Gang Wang, Hideyuki Ujiie, Akihiko Shibaki, Wataru Nishie, Yasuki Tateishi, Kazuhiro Kikuchi, Qiang Li, James R. McMillan, Hiroshi Morioka, Daisuke Sawamura, Hideki Nakamura, Hiroshi Shimizu
    AMERICAN JOURNAL OF PATHOLOGY, 176, 2, 914, 925, Feb. 2010, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • A Novel Active Mouse Model for Bullous Pemphigoid Targeting Humanized Pathogenic Antigen
    Hideyuki Ujiie, Akihiko Shibaki, Wataru Nishie, Daisuke Sawamura, Gang Wang, Yasuki Tateishi, Qiang Li, Reine Moriuchi, Hongjiang Qiao, Hideki Nakamura, Masashi Akiyama, Hiroshi Shimizu
    JOURNAL OF IMMUNOLOGY, 184, 4, 2166, 2174, Feb. 2010, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Hereditary Benign Telangiectasia: Two Families With Punctate Telangiectasias Surrounded by Anemic Halos
    Hideyuki Ujiie, Kazuo Kodama, Masashi Akiyama, Hiroshi Shimizu
    ARCHIVES OF DERMATOLOGY, 146, 1, 98, 99, Jan. 2010, [Peer-reviewed], [International Magazine]
    English
  • Successful Treatment of Nail Lichen Planus with Topical Tacrolimus
    Hideyuki Ujiie, Akihiko Shibaki, Masashi Akiyama, Hiroshi Shimizu
    ACTA DERMATO-VENEREOLOGICA, 90, 2, 218, 219, 2010, [Peer-reviewed], [International Magazine]
    English
  • A Novel Humanized Neonatal Autoimmune Blistering Skin Disease Model Induced by Maternally Transferred Antibodies
    Wataru Nishie, Daisuke Sawamura, Ken Natsuga, Satoru Shinkuma, Maki Goto, Akihiko Shibaki, Hideyuki Ujiie, Edit Olasz, Kim B. Yancey, Hiroshi Shimizu
    JOURNAL OF IMMUNOLOGY, 183, 6, 4088, 4093, Sep. 2009, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Bloody Nipple Discharge in an Infant
    Hideyuki Ujiie, Masashi Akiyama, Rinko Osawa, Satoru Shida, Satoru Aoyagi, Hiroshi Shimizu
    ARCHIVES OF DERMATOLOGY, 145, 9, 1068, 1069, Sep. 2009, [Peer-reviewed], [International Magazine]
    English
  • Conradi-Hunermann-Happle syndrome with abnormal lamellar granule contents.
    Akiyama M, Sakai K, Hayasaka K, Tabata N, Yamada M, Ujiie H, Shibaki A, Shimizu H
    Br J Dermatol, 160, 1335, 1337, 2009, [Peer-reviewed]
  • A novel PTPN11 missense mutation in a patient with LEOPARD syndrome.
    Osawa R, Akiyama M, Yamanaka Y, Ujiie H, Nemoto-Hasebe I, Takeda A, Yanagi T, Shimizu H
    Br J Dermatol, 161, 1202, 1204, 2009, [Peer-reviewed]
  • Harlequin ichthyosis model mouse reveals alveolar collapse and severe fetal skin barrier defects
    Teruki Yanagi, Masashi Akiyama, Hiroshi Nishihara, Kaori Sakai, Wataru Nishie, Shinya Tanaka, Hiroshi Shimizu
    HUMAN MOLECULAR GENETICS, 17, 19, 3075, 3083, Oct. 2008, [Peer-reviewed]
    English, Scientific journal
  • Leukaemic dissemination of Merkel cell carcinoma in a patient with systemic lupus erythematosus.
    Nemoto I, Sato-Matsumura KC, Fujita Y, Natsuga K, Ujiie H, Tomita Y, Kato N, Kondo M, Ohnishi K
    Clinical and experimental dermatology, 33, 3, 270, 272, 3, May 2008, [Peer-reviewed]
    Nemoto I, Sato-Matsumura KC, Fujita Y, Natsuga K, Ujiie H, Tomita Y, Kato N, Kondo M, Ohnishi K, Clinical and experimental dermatology, 2008, vol. 33, no. 3, pp. 270-272
  • Large subcutaneous abscesses caused by Mycobacterium fortuitum infection
    Reine Moriuchi, Ken Arita, Hideyuki Ujiie, Kazuo Kodama, Tadamichi Shimizu, Hiroshi Shimizu
    ACTA DERMATO-VENEREOLOGICA, 88, 3, 313, 314, 2008, [Peer-reviewed], [International Magazine]
    English
  • Novel ABCA12 mutations identified in two cases of non-bullous congenital ichthyosiform erythroderma associated with multiple skin malignant neoplasia
    Ken Natsuga, Masashi Akiyama, Naoko Kato, Kaori Sakai, Yoriko Sugiyama-Nakagiri, Machiko Nishimura, Hiroo Hata, Masataka Abe, Ken Arita, Yukiko Tsuji-Abe, Takashi Onozuka, Satoru Aoyagi, Kazuo Kodama, Hideyuki Ujiie, Yuki Tomita, Hiroshi Shimizu
    JOURNAL OF INVESTIGATIVE DERMATOLOGY, 127, 11, 2669, 2673, Nov. 2007, [Peer-reviewed], [International Magazine]
    English
  • Non-Hodgkin lymphoma preceded by recalcitrant eczema
    Ken Natsuga, Riichiro Abe, Hideyuki Ujiie, Akihiko Shibaki, Daisuke Sawamura, Mitsufumi Nishio, Katsuya Fujimoto, Takao Koike, Hiroshi Shimizu
    EUROPEAN JOURNAL OF HAEMATOLOGY, 79, 4, 369, 370, Oct. 2007, [Peer-reviewed], [International Magazine]
    English, Scientific journal
  • Keratoacanthoma developing on nevus sebaceous in a child
    Hideyuki Ujiie, Naoko Kato, Ken Natsuga, Yuki Tomita
    JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 56, 2, S57, S58, Feb. 2007, [Peer-reviewed], [International Magazine]
    English
  • Severe cholinergic urticaria successfully treated with scopolamine butylbromide in addition to antihistamines.
    Ujiie H, Shimizu T, Natsuga K, Arita K, Tomizawa K, Shimizu H
    Clinical and experimental dermatology, Jul. 2006, [Peer-reviewed]
  • Peginterferon alfa-2b for mycosis fungoides
    T Yanagi, T Shimizu, H Ujiie, M Ito, R Abe, Y Tsuji-Abe, S Hige, H Shimizu
    ARCHIVES OF DERMATOLOGY, 142, 5, 649, 651, May 2006, [Peer-reviewed], [International Magazine]
    English
  • Development of lichen planus and psoriasis on lesions of vitiligo vulgaris.
    Ujiie H, Sawamura D, Shimizu H
    Clinical and experimental dermatology, 31, 375, 377, 3, May 2006, [Peer-reviewed]
  • Lupus erythematosus profundus successfully treated with dapsone: Review of the literature
    H Ujiie, T Shimizu, M Ito, K Arita, H Shimizu
    ARCHIVES OF DERMATOLOGY, 142, 3, 399, 401, Mar. 2006, [Peer-reviewed], [International Magazine]
    English
  • Cutaneous reactions to imatinib mesylate treated by topical steroid
    H Ujiie, T Shimizu, H Shimizu
    AMERICAN JOURNAL OF HEMATOLOGY, 78, 3, 246, 246, Mar. 2005, [Peer-reviewed], [International Magazine]
    English
  • Prolonged dermatitis distant to the site of squaric acid dibutyl ester applications and recovery of alopecia areata.
    Ujiie H, Sawamura D, Shibaki A, Shimizu H
    Clin Exp Dermatol, 30, 579, 580, 2005, [Peer-reviewed]
  • Intractable erythema nodosum associated with severe breast abscesses: reports of two cases.
    Ujiie H, Sawamura D, Yokota K, Tateishi Y, Inokuma D, Shimizu H
    Clin Exp Dermatol, 30, 584, 585, 2005, [Peer-reviewed]
  • Pyoderma gangrenosum associated with Takayasu's arteritis.
    Ujiie H, Sawamura D, Yokota K, Nishie W, Shichinohe R, Shimizu H
    Clin Exp Dermatol, 29, 4, 357, 359, 2004, [Peer-reviewed], [International Magazine]
    English, Scientific journal, Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by destructive, necrotizing and noninfective ulceration of the skin mostly on lower extremities. PG is well known as a complication of Takayasu's arteritis in Japan. However, this association is not commonly observed in North American and European patients. We describe a case of PG that was associated with Takayasu's arteritis who was successfully treated with systemic cyclosporin. We have reviewed 35 well-documented PG cases with Takayasu's arteritis in comparison to 106 PG cases without Takayasu's arteritis. The results demonstrate that this association occurs predominantly in young females and that these cases exhibit more widespread PG lesions.
  • Generalized pustular psoriasis and Hepatitis C virus infection.
    Ujiie H, Shimizu T, Shimizu H
    Acta virologica, Jan. 2004, [Peer-reviewed]

Other Activities and Achievements

  • 電子照射後にMilia en plaqueを生じた菌状息肉症の1例               
    川村 拓也, 山口 泰之, 片山 奨, 藤村 悠, 泉 健太郎, 志藤 元泰, 橋本 孝之, 氏家 英之, 日本臨床皮膚科医会雑誌, 41, 2, 367, 367, Apr. 2024
    日本臨床皮膚科医会, Japanese
  • 【水疱をきたす疾患】多彩な臨床像を呈する自己免疫性水疱症
    氏家 英之, 皮膚病診療, 46, 3, 208, 212, Mar. 2024
    (株)協和企画, Japanese
  • エルロチニブとラムシルマブの併用療法中に穿孔性皮膚症と紫斑,皮膚潰瘍を生じた1例
    森田 裕介, 今福 恵輔, 板本 想太, 挽地 史織, 眞井 翔子, 宮澤 元, 得地 景子, 榊原 純, 氏家 英之, 臨床皮膚科, 78, 2, 119, 124, Feb. 2024
    (株)医学書院, Japanese
  • 左母趾に生じたOnychomatricoma(爪母腫)の1例               
    澁佐 知歩, 前田 拓哉, 小住 英之, 椎谷 千尋, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 134, 1, 99, 99, Jan. 2024
    (公社)日本皮膚科学会, Japanese
  • 骨髄異形成症候群(MDS)に合併した成人発症の骨髄性プロトポルフィリン症の1例               
    島野 麻由奈, 泉 健太郎, 澁佐 知歩, 濱 芙美, 三浦 譲司, 成瀬 早紀, 劉 柱亭, 眞井 洋輔, 氏家 英之, 小林 景樹, 日本皮膚科学会雑誌, 134, 1, 100, 100, Jan. 2024
    (公社)日本皮膚科学会, Japanese
  • 耳介変形を伴った壊疽性膿皮症の1例               
    成瀬 早紀, 渡邉 美佳, 眞井 洋輔, 夏賀 健, 氏家 英之, 日本皮膚科学会雑誌, 134, 1, 100, 100, Jan. 2024
    (公社)日本皮膚科学会, Japanese
  • 抗BP230抗体単独陽性の類天疱瘡               
    氏家 英之, 日本皮膚科学会雑誌, 134, 1, 104, 105, Jan. 2024
    (公社)日本皮膚科学会, Japanese
  • 患者汗を利用した好塩基球活性化試験がアレルゲンの同定に有用であった自己汗アナフィラキシーの1例               
    泉 健太郎, 山口 泰之, 平田 悠, 柳 輝希, 夏賀 健, 氏家 英之, 日本皮膚免疫アレルギー学会総会学術大会プログラム・抄録集, 53回, 219, 219, Dec. 2023
    (一社)日本皮膚免疫アレルギー学会, Japanese
  • Generalized basaloid follicular hamartoma syndromeの長期経過               
    澁佐 知歩, 渡邉 美佳, 夏賀 健, 氏家 英之, Hsu Chao-Kai, 日本皮膚科学会雑誌, 133, 12, 2853, 2853, Nov. 2023
    (公社)日本皮膚科学会, Japanese
  • 慢性骨髄単球性白血病に合併した尋常性天疱瘡の1例               
    三浦 譲司, 眞井 洋輔, 成瀬 早紀, 澁佐 知歩, 島野 麻由奈, 野原 拓馬, 挽地 史織, 小住 英之, 長田 悠理, 黒澤 卓, 眞井 翔子, 宮澤 元, 得地 景子, 渡邉 美佳, 今福 恵輔, 片山 奨, 氏家 韻欣, 泉 健太郎, 氏家 英之, 宮澤 仁, 遠藤 知之, 日本皮膚科学会雑誌, 133, 12, 2854, 2854, Nov. 2023
    (公社)日本皮膚科学会, Japanese
  • ニボルマブによる術後補助療法中に膀胱炎を生じた悪性黒色腫の1例               
    濱 芙美, 前田 拓哉, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 133, 12, 2855, 2855, Nov. 2023
    (公社)日本皮膚科学会, Japanese
  • 【アトピー性皮膚炎の診療に必要な最新知識】アトピー性皮膚炎の病態 バリア機能障害               
    氏家 英之, アレルギーの臨床, 43, 8, 583, 586, Aug. 2023
    (株)北隆館, Japanese
  • 四酸化オスミウムによる化学熱傷により色素沈着を生じた1例               
    成瀬 早紀, 高島 翔太, 夏賀 健, 氏家 英之, 日本皮膚科学会雑誌, 133, 9, 2182, 2182, Aug. 2023
    (公社)日本皮膚科学会, Japanese
  • 抗真菌薬外用により急速に上皮化した難治性うっ滞性皮膚潰瘍の1例               
    澁佐 知歩, 宮澤 元, 渡邉 美佳, 黒澤 卓, 長田 悠里, 眞井 翔子, 得地 景子, 今福 恵輔, 高島 翔太, 氏家 英之, 細川 一義, 日本皮膚科学会雑誌, 133, 9, 2183, 2183, Aug. 2023
    (公社)日本皮膚科学会, Japanese
  • 多彩な臨床像を呈する自己免疫性水疱症               
    氏家 英之, 日本皮膚科学会雑誌, 133, 5, 1315, 1315, May 2023
    (公社)日本皮膚科学会, Japanese
  • 当科における巻き爪マイスターによる治療経験               
    瀬尾 拓志, 椎谷 千尋, 小住 英之, 氏家 英之, 日本皮膚科学会雑誌, 133, 5, 1381, 1381, May 2023
    (公社)日本皮膚科学会, Japanese
  • 当科におけるポリヘキサニド・ベタイン溶液を用いた局所療法の使用経験               
    須貝 達朗, 今福 恵輔, 板本 想太, 挽地 史織, 黒澤 卓, 長田 悠里, 葭本 倫大, 森田 裕介, 氏家 英之, 日本皮膚科学会雑誌, 133, 5, 1403, 1403, May 2023
    (公社)日本皮膚科学会, Japanese
  • エルロチニブ(EGFRチロシンキナーゼ阻害薬)により生じた血管炎の1例               
    挽地 史織, 宮澤 元, 板本 想太, 眞井 翔子, 得地 景子, 今福 恵輔, 榊原 純, 氏家 英之, 日本臨床皮膚科医会雑誌, 40, 3, 466, 466, May 2023
    日本臨床皮膚科医会, Japanese
  • アトピー性皮膚炎に合併した難治性の円形脱毛症に対してデュピルマブからバリシチニブへの変更が奏功した1例               
    瀬尾 拓志, 泉 健太郎, 川村 拓也, 辻脇 真澄, 氏家 英之, 日本皮膚科学会雑誌, 133, 4, 707, 707, Apr. 2023
    (公社)日本皮膚科学会, Japanese
  • 右耳介後部の結節               
    中里 信一, 外丸 詩野, 清水 亜衣, 高桑 恵美, 大塚 紀幸, 前田 拓哉, 今福 恵輔, 阿南 隆, 氏家 英之, 松野 吉宏, 日本皮膚病理組織学会抄録集, 39回, 40,22, 40,22, Apr. 2023
    日本皮膚病理組織学会, Japanese
  • 【フレッシャーズ特集:皮膚科の基本マニュアル】(Part1)診療の基本 蛍光抗体 基本+α               
    片山 奨, 氏家 英之, Visual Dermatology, 22, 4, 353, 355, Mar. 2023
    (株)Gakken, Japanese
  • 表皮基底膜部ネイティブタンパク質複合体を利用した新規ELISA法は粘膜類天疱瘡自己抗体検出に有用である               
    眞井 翔子, 泉 健太郎, 眞井 洋輔, 夏賀 健, 氏家 英之, 石井 文人, 澤村 大輔, Franziska Schauer, Dimitra Kiritsi, 西江 渉, 日本皮膚科学会雑誌, 133, 3, 539, 539, Mar. 2023
    (公社)日本皮膚科学会, Japanese
  • ポリヘキサニド・ベタイン溶液を用いた局所療法が奏効した下腿潰瘍の2例               
    須貝 達朗, 今福 恵輔, 板本 想太, 挽地 史織, 葭本 倫大, 森田 裕介, 氏家 英之, 日本皮膚科学会雑誌, 133, 3, 540, 540, Mar. 2023
    (公社)日本皮膚科学会, Japanese
  • 表皮水疱症に合併したacantholytic acanthomaの組織学的検討               
    板本 想太, 夏賀 健, 高島 翔太, 氏家 英之, 日本皮膚科学会雑誌, 133, 3, 540, 540, Mar. 2023
    (公社)日本皮膚科学会, Japanese
  • エルロチニブ(EGFR阻害薬)により発症した薬剤性皮膚潰瘍の1例               
    挽地 史織, 宮澤 元, 板本 想太, 眞井 翔子, 得地 景子, 今福 恵輔, 氏家 英之, 榊原 純, 日本皮膚科学会雑誌, 133, 3, 540, 540, Mar. 2023
    (公社)日本皮膚科学会, Japanese
  • 日本皮膚科学会ガイドライン 類天疱瘡(後天性表皮水疱症を含む)診療ガイドライン補遺版
    青山 裕美, 杉山 聖子, 山上 淳, 高橋 勇人, 岩田 浩明, 名嘉眞 武國, 池田 志斈, 石井 文人, 黒沢 美智子, 澤村 大輔, 鶴田 大輔, 天谷 雅行, 氏家 英之, 日本皮膚科学会類天疱瘡(後天性表皮水疱症を含む)診療ガイドライン策定委員会, 日本皮膚科学会雑誌, 133, 2, 189, 193, Feb. 2023
    (公社)日本皮膚科学会, Japanese
  • 表皮融解性魚鱗癬に生じたclear cell acanthomaの1例               
    秦 玉瑩, 宮内 俊成, 椎谷 千尋, 鈴木 翔太朗, 夏賀 健, 氏家 英之, 乃村 俊史, 日本皮膚科学会雑誌, 133, 2, 268, 268, Feb. 2023
    (公社)日本皮膚科学会, Japanese
  • 表皮水疱症に合併した血管腫の2例               
    板本 想太, 夏賀 健, 高島 翔太, 氏家 英之, 日本皮膚科学会雑誌, 133, 2, 272, 272, Feb. 2023
    (公社)日本皮膚科学会, Japanese
  • 当院における乾癬性ぶどう膜炎4例のまとめ               
    宮澤 元, 夏賀 健, 今福 恵輔, 氏家 英之, 南場 研一, 日本皮膚科学会雑誌, 133, 2, 273, 273, Feb. 2023
    (公社)日本皮膚科学会, Japanese
  • Annular leukocytoclastic vasculitisの1例               
    長田 悠里, 宮澤 元, 板本 想太, 挽地 史織, 眞井 翔子, 今福 恵輔, 得地 景子, 泉 健太郎, 氏家 英之, 日本皮膚科学会雑誌, 133, 2, 274, 274, Feb. 2023
    (公社)日本皮膚科学会, Japanese
  • LMNA遺伝子のスプライス部位に変異を同定した重症型Hutchison-Gilford Progeria症候群の1症例               
    外木 秀文, 川端 むつみ, 高橋 伸浩, 片山 奨, 氏家 英之, 山田 茉未子, 鈴木 寿人, 小崎 健次郎, 日本小児科学会雑誌, 127, 2, 251, 251, Feb. 2023
    (公社)日本小児科学会, Japanese
  • 体重減少は悪性黒色腫の予後不良因子になりうる
    田中有沙, 田中有沙, 宮澤元, 前田拓哉, 北村真也, 柳輝希, 氏家英之, 西日本皮膚科, 85, 3, 2023
  • 姑息的切除を行った乳房外パジェット病の予後に関する検討
    瀬尾拓志, 北村真也, 柳輝希, 前田拓哉, 氏家英之, 加齢皮膚医学セミナー, 18, 1, 37, 37, 2023
    加齢皮膚医学研究会, Japanese
  • 水疱性類天疱瘡とBullous Rheumatoid Neutrophilic Dermatosisとの鑑別を要したリウマトイド血管炎
    田中有沙, 田中有沙, 柳輝希, 今福恵輔, 渥美達也, 氏家英之, 皮膚科の臨床, 65, 10, 2023
  • アトピー性皮膚炎患者におけるViewアレルギー39検査結果の検証およびアレルギーに関するアンケート調査結果の報告               
    辻脇 真澄, 泉 健太郎, 川村 拓也, 氏家 英之, 日本皮膚免疫アレルギー学会総会学術大会プログラム・抄録集, 52回, 187, 187, Dec. 2022
    (一社)日本皮膚免疫アレルギー学会, Japanese
  • 【壊疽性膿皮症】壊疽性膿皮症の併存症と診療科間連携               
    氏家 英之, 皮膚科, 2, 5, 602, 608, Nov. 2022
    (有)科学評論社, Japanese
  • DNA複製ストレス反応の変化が毛孔性紅色粃糠疹5型の自然治癒現象を誘導する               
    宮内 俊成, 鈴木 翔多朗, 竹田 真依, Teng Peh Jin, 相庭 昌之, 夏賀 健, 藤田 靖幸, 清水 宏, 氏家 英之, 坂本 泰子, 武市 拓也, 秋山 真志, 乃村 俊史, 日本皮膚科学会雑誌, 132, 12, 2695, 2695, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 間歇的空気圧迫装置使用後に両下腿に"ヒト型"の接触皮膚炎を生じた1例               
    瀬尾 拓志, 宮内 俊成, 氏家 英之, 河上 強志, 日本皮膚科学会雑誌, 132, 12, 2695, 2695, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 基底膜部にIgM抗体の沈着を認めた表皮下水疱症の1例               
    平野 瑶子, 岩田 浩明, 辻脇 真澄, 眞井 翔子, 眞井 洋輔, 今福 恵輔, 泉 健太郎, 氏家 英之, 古賀 浩嗣, 日本皮膚科学会雑誌, 132, 12, 2696, 2696, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 先行する紅皮症と好酸球増多からIgA腎症を同定した1例               
    高津 三奈, 夏賀 健, 氏家 英之, 八反田 文彦, 日本皮膚科学会雑誌, 132, 12, 2696, 2696, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • Wnt/β-cateninシグナルが表皮角化細胞のヘミデスモソームを制御する               
    小住 英之, 野原 拓馬, 藤村 悠, 岩田 浩明, 中村 秀樹, 氏家 英之, 夏賀 健, 渡邉 美佳, Donati Giacomo, 新熊 悟, 築山 忠維, 日本皮膚科学会雑誌, 132, 12, 2697, 2697, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 染毛剤のエチドロン酸により生じたerythema multiforme-like contact dermatitis(EMCD)の1例               
    長谷 恭宏, 小住 英之, 織田 晃央, 宮澤 元, 得地 景子, 氏家 英之, 日本皮膚科学会雑誌, 132, 12, 2698, 2699, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 間擦部に色素性病変を認めたlichen planus pigmentosus-inversus(LPPI)の1例               
    劉 柱亨, 岩田 浩明, 北村 真也, 氏家 英之, 大口 由香, 日本皮膚科学会雑誌, 132, 12, 2699, 2699, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • ネシツムマブによる後天性穿孔性皮膚症の1例               
    木村 彩萌, 小住 英之, 夏賀 健, 氏家 英之, 合田 智宏, 日本皮膚科学会雑誌, 132, 12, 2699, 2699, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 乾癬患者におけるTNF-α阻害薬使用で血清KL-6が上昇する               
    瀬尾 拓志, 宮澤 元, 岩田 浩明, 夏賀 健, 氏家 英之, 日本皮膚科学会雑誌, 132, 12, 2700, 2700, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 高リコピン血症に伴う皮膚柑皮症の1例               
    田中 有沙, 宮内 俊成, 北村 真也, 岩田 浩明, 氏家 英之, 秦 洋郎, 日本皮膚科学会雑誌, 132, 12, 2700, 2700, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 日本人の乾癬性関節炎(PsA)は肥満と正の相関を示す 96例の横断研究               
    山賀 三紗子, 宮澤 元, 瀬尾 拓志, 田中 有沙, 岩田 浩明, 夏賀 健, 氏家 英之, 日本皮膚科学会雑誌, 132, 12, 2701, 2701, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 患側の萎縮を来したinverse Klippel-Trenaunay syndromeの1例               
    長谷 恭宏, 宮内 俊成, 氏家 英之, 加藤 将, 日本皮膚科学会雑誌, 132, 12, 2701, 2701, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 水疱性類天疱瘡における低ナトリウム血症の有病率に関する検討               
    板本 想太, 眞井 洋輔, 葭本 倫大, 氏家 韻欣, 泉 健太郎, 氏家 英之, 日本皮膚科学会雑誌, 132, 12, 2702, 2702, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 盲腸皮膚瘻の1例               
    野原 拓馬, 片山 奨, 氏家 英之, 山口 泰之, 田中 聡一, 小田 陽一郎, 廣瀬 和幸, 中村 裕之, 日本皮膚科学会雑誌, 132, 12, 2703, 2703, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • 治療に難渋したペムブロリズマブによる中毒性表皮壊死症の1例               
    長田 悠里, 葭本 倫大, 黒澤 卓, 瀬尾 拓志, 須貝 達朗, 森田 裕介, 今福 恵輔, 氏家 韻欣, 氏家 英之, 山村 貴洋, 日本皮膚科学会雑誌, 132, 12, 2705, 2705, Nov. 2022
    (公社)日本皮膚科学会, Japanese
  • Diagnosis and Management of Porocarcinoma
    Kodai Miyamoto, Teruki Yanagi, Takuya Maeda, Hideyuki Ujiie, Cancers, 14, 21, Nov. 2022, [International Magazine]
    English
  • 【皮膚疾患研究の最前線】水疱性類天疱瘡の基礎研究最前線               
    氏家 英之, BIO Clinica, 37, 9, 816, 821, Aug. 2022
    (株)北隆館, Japanese
  • 加齢に着目した新たな自己抗体産生機序の解明               
    氏家 英之, 高木賞研究結果報告書集, 3回, 43, 45, Jul. 2022
    (公財)マルホ・高木皮膚科学振興財団, Japanese
  • 表皮融解性魚鱗癬の1家系               
    坂本 想太, 宮内 俊成, 眞井 洋輔, 氏家 英之, 乃村 俊史, 角化症研究会記録集, 36, 11, 15, Jun. 2022
    角化症研究会事務局, Japanese
  • 新規CARD14変異を同定した自己炎症性角化症の姉弟例               
    宮内 俊成, 岩田 浩明, 羽賀 直哉, Peh Jin Teng, 夏賀 健, 氏家 英之, 小林 仁, 乃村 俊史, 角化症研究会記録集, 36, 66, 70, Jun. 2022
    角化症研究会事務局, Japanese
  • GJB2変異を同定した先天性感音性難聴を伴う掌蹠角化症の家族例               
    木村 彩萌, 宮内 俊成, Peh Jin Teng, 柳 輝希, 長谷川 聡, 氏家 英之, 西日本皮膚科, 84, 3, 265, 265, Jun. 2022
    日本皮膚科学会-西部支部, Japanese
  • CDK4/6阻害剤は乳房外Paget病の腫瘍増殖を抑制する               
    北村 真也, 前田 拓哉, 柳 輝希, 氏家 英之, 日本皮膚悪性腫瘍学会学術大会プログラム・抄録集, 38回, 154, 154, May 2022
    (一社)日本皮膚悪性腫瘍学会, Japanese
  • 壊疽性膿皮症の診断と治療戦略               
    氏家 英之, 日本皮膚科学会雑誌, 132, 5, 1280, 1280, May 2022
    (公社)日本皮膚科学会, Japanese
  • 乾癬患者における爪所見と肥満の関連性を評価する横断的研究               
    宮澤 元, 山賀 三紗子, 田中 有沙, 瀬尾 拓志, 岩田 浩明, 夏賀 健, 氏家 英之, 日本皮膚科学会雑誌, 132, 5, 1309, 1309, May 2022
    (公社)日本皮膚科学会, Japanese
  • 中枢性免疫寛容に着目した自己免疫性水疱症の発症機序の解明               
    氏家 英之, 日本皮膚科学会雑誌, 132, 5, 1378, 1378, May 2022
    (公社)日本皮膚科学会, Japanese
  • 肺病変にステロイドが奏功したPOIKTMPの1例(アンコール演題)
    佐藤 理子, 鈴木 雅, 猪狩 智生, 中村 友彦, 高橋 桂, 佐々木 真知子, 木村 孔一, 木村 裕樹, 宮内 俊成, 乃村 俊史, 氏家 英之, 山口 直子, 大塚 紀幸, 外丸 詩野, 今野 哲, 日本呼吸器学会誌, 11, 増刊, 272, 272, Apr. 2022
    (一社)日本呼吸器学会, Japanese
  • 肺病変にステロイドが奏功したPOIKTMPの1例(アンコール演題)               
    佐藤 理子, 鈴木 雅, 猪狩 智生, 中村 友彦, 高橋 桂, 佐々木 真知子, 木村 孔一, 木村 裕樹, 宮内 俊成, 乃村 俊史, 氏家 英之, 山口 直子, 大塚 紀幸, 外丸 詩野, 今野 哲, 日本呼吸器学会誌, 11, 増刊, 272, 272, Apr. 2022
    (一社)日本呼吸器学会, Japanese
  • 【後期研修医が知っておくべき皮膚科診療3つのポイント】(Part2.)皮膚疾患の基本(解説12) 後期研修医が知っておくべき「水疱性類天疱瘡」3つのポイント               
    氏家 英之, Visual Dermatology, 21, 4, 384, 388, Mar. 2022
    (株)Gakken, Japanese
  • 類天疱瘡Update               
    氏家 英之, 日本皮膚科学会雑誌, 132, 3, 494, 494, Mar. 2022
    (公社)日本皮膚科学会, Japanese
  • 難治性の重症Hailey-Hailey病にアプレミラストが著効した1例               
    山賀 三紗子, 宮内 俊成, Peh Jin Teng, 岩田 浩明, 氏家 英之, 乃村 俊史, 日本皮膚科学会雑誌, 132, 3, 510, 510, Mar. 2022
    (公社)日本皮膚科学会, Japanese
  • 薬剤投与に伴う水疱性類天疱瘡               
    氏家 英之, 日本皮膚科学会雑誌, 132, 2, 291, 291, Feb. 2022
    (公社)日本皮膚科学会, Japanese
  • 類天疱瘡の最新知見               
    氏家 英之, 日本皮膚科学会雑誌, 132, 2, 339, 339, Feb. 2022
    (公社)日本皮膚科学会, Japanese
  • 経口避妊薬関連性unilateral nevoid telangiectasiaの1例               
    野原 拓馬, 夏賀 健, 氏家 英之, 安岡 厚, 日本皮膚科学会雑誌, 132, 2, 356, 356, Feb. 2022
    (公社)日本皮膚科学会, Japanese
  • CARD14変異に伴う炎症経路の解析とそれに基づく治療が奏功した1家系               
    宮内 俊成, 岩田 浩明, 羽賀 直哉, Peh Jin Teng, 夏賀 健, 氏家 英之, 小林 仁, 乃村 俊史, 日本皮膚科学会雑誌, 132, 1, 88, 88, Jan. 2022
    (公社)日本皮膚科学会, Japanese
  • 心房中隔欠損症に対する経カテーテル的欠損孔閉鎖術後に軽快した掌蹠型乾癬の1例               
    須貝 達朗, 岩田 浩明, 氏家 英之, 中村 裕之, 廣上 貢, 齊院 康平, 岩野 弘幸, 日本皮膚科学会雑誌, 132, 1, 88, 89, Jan. 2022
    (公社)日本皮膚科学会, Japanese
  • アダリムマブによる病勢コントロールの後、二期的再建を実施した化膿性汗腺炎の1例               
    織田 晃史, 前田 拓哉, 北村 真也, 柳 輝希, 岩田 浩明, 氏家 英之, 日本皮膚科学会雑誌, 132, 1, 94, 94, Jan. 2022
    (公社)日本皮膚科学会, Japanese
  • 私の視点 自己抗体測定の落とし穴
    氏家 英之, 皮膚病診療, 43, 12, 1154, 1154, Dec. 2021
    (株)協和企画, Japanese
  • 【あらためて学ぶ。薬疹と皮膚障害】知っておくべき薬物障害 DPP-4阻害薬関連水疱性類天疱瘡
    氏家 英之, 皮膚科の臨床, 63, 12, 1794, 1800, Nov. 2021
    金原出版(株), Japanese
  • 【目標を見据えた高齢者糖尿病管理~実態の理解から薬物療法まで~】高齢者薬物療法の注意点 糖尿病薬における皮膚合併症               
    氏家 英之, 月刊糖尿病, 13, 11, 80, 85, Nov. 2021
    (株)医学出版, Japanese
  • 間歇的空気圧迫装置使用時に着用するコンフォートスリーブで接触皮膚炎を生じた1例               
    瀬尾 拓志, 宮内 俊成, 河上 強志, 氏家 英之, 日本皮膚免疫アレルギー学会総会学術大会プログラム・抄録集, 51回, 226, 226, Nov. 2021
    (一社)日本皮膚免疫アレルギー学会, Japanese
  • 【エキスパートから学ぶ「再発抑制・寛解維持に悩む疾患」】(Part2.)自己免疫性水疱症(opinion 5-1) 水疱性類天疱瘡 私の考え方(1)               
    氏家 英之, Visual Dermatology, 20, 11, 1148, 1149, Oct. 2021
    (株)Gakken, Japanese
  • 表皮下水疱は毛包の成長を犠牲にして治癒する               
    藤村 悠, 高島 翔太, 中村 秀樹, 小住 英之, 王 禹楠, 眞井 洋輔, 氏家 英之, 岩田 浩明, 西江 渉, 清水 宏, 夏賀 健, 渡邉 美佳, 大野 航太, 小林 康明, 長山 雅晴, Andrea Lauria, Valentina Proserpio, Salvatore Oliviero, Giacomo Donati, 日本皮膚科学会雑誌, 131, 9, 2071, 2071, Aug. 2021
    (公社)日本皮膚科学会, Japanese
  • 当科で加療した血管肉腫21例の臨床的検討               
    瀬尾 拓志, 北村 真也, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 131, 9, 2071, 2071, Aug. 2021
    (公社)日本皮膚科学会, Japanese
  • 家族間で異なる臨床を呈したCARD14変異を有する炎症性角化症の3例               
    岩田 浩明, 羽賀 直哉, 宮内 俊成, Teng Peh Jin, 氏家 英之, 小林 仁, 日本皮膚科学会雑誌, 131, 8, 1893, 1893, Jul. 2021
    (公社)日本皮膚科学会, Japanese
  • 【皮膚科医もいまさらしっかり総復習!糖尿病の今!】(Part2)糖尿病の皮膚!いま改めて総復習!(Atlas 9) DPP-4阻害薬を投薬中に発症した水疱性類天疱瘡               
    氏家 英之, Visual Dermatology, 20, 8, 822, 822, Jul. 2021
    (株)Gakken, Japanese
  • 【皮膚科医もいまさらしっかり総復習!糖尿病の今!】(Part2)糖尿病の皮膚!いま改めて総復習!(Atlas 9) DPP-4阻害薬を投薬中に発症した水疱性類天疱瘡               
    氏家 英之, Visual Dermatology, 20, 8, 822, 822, Jul. 2021
    (株)学研メディカル秀潤社, Japanese
  • VandetanibはEGFR阻害作用により皮膚有棘細胞癌の増殖を抑制する               
    北村 真也, 前田 拓哉, 柳 輝希, 氏家 英之, 日本皮膚悪性腫瘍学会学術大会プログラム・抄録集, 37回, 169, 169, Jun. 2021
    (一社)日本皮膚悪性腫瘍学会, Japanese
  • 類天疱瘡の病態と最新治療               
    氏家 英之, 日本皮膚科学会雑誌, 131, 5, 1175, 1175, May 2021
    (公社)日本皮膚科学会, Japanese
  • その他の自己免疫性水疱症の治療               
    氏家 英之, 日本皮膚科学会雑誌, 131, 5, 1255, 1255, May 2021
    (公社)日本皮膚科学会, Japanese
  • 両下肢にびまん性に生じた増殖性天疱瘡               
    川村 拓也, 須貝 達朗, 宮内 俊成, 村松 憲, 古賀 浩嗣, 石井 文人, 泉 健太郎, 氏家 英之, 日本皮膚科学会雑誌, 131, 5, 1397, 1397, May 2021
    (公社)日本皮膚科学会, Japanese
  • HRAS変異を有する表皮母斑上に生じた多発有棘細胞癌               
    柳 輝希, 北村 真也, 前田 拓哉, 奈良平 敦司, 氏家 英之, 西原 広史, 慶応義塾大学医学部腫瘍センターゲノム医療ユニット, 日本皮膚科学会雑誌, 131, 5, 1404, 1404, May 2021
    (公社)日本皮膚科学会, Japanese
  • IgA血管炎に類似した臨床、病理組織像を呈したトコジラミ刺症の1例               
    織田 晃央, 宮内 俊成, 氏家 韻欣, 氏家 英之, 日本皮膚科学会雑誌, 131, 3, 539, 539, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • 免疫チェックポイント阻害薬投与に伴い類天疱瘡を発症した3例               
    葭本 倫大, 織田 晃央, 川村 拓也, 須貝 達朗, 羽賀 直哉, 藤村 悠, 氏家 韻欣, 泉 健太郎, 氏家 英之, 田口 純, 松村 和子, 藤田 昭久, 木村 有貴, 日本皮膚科学会雑誌, 131, 3, 540, 540, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • 抗デスモコリン抗体が検出された増殖性天疱瘡の1例               
    川村 拓也, 織田 晃央, 眞井 洋輔, 須貝 達朗, 羽賀 直哉, 藤村 悠, 氏家 英之, 古賀 浩嗣, 石井 文人, 日本皮膚科学会雑誌, 131, 3, 540, 540, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • VandetanibはEGFR阻害作用により皮膚有棘細胞癌の増殖を抑制する               
    北村 真也, 前田 拓哉, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 131, 3, 541, 541, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • デノスマブにより1年間病勢の進行を抑制した血管肉腫の1例               
    前田 拓哉, 北村 真也, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 131, 3, 541, 541, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • HPV78が検出された扁平疣贅の1例               
    島野 麻由奈, 宮内 俊成, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 131, 3, 541, 541, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • 選択的血漿交換療法を施行した難治性水疱性類天疱瘡の2例               
    葭本 倫大, 須貝 達朗, 川村 拓也, 島野 麻由奈, 宮本 航大, 眞井 洋輔, 羽賀 直哉, 藤村 悠, 宮内 俊成, 氏家 韻欣, 泉 健太郎, 氏家 英之, 中沢 大悟, 日本皮膚科学会雑誌, 131, 3, 542, 542, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • Hereditary leiomyomatosis and renal cell cancer(HLRCC)と診断した1例               
    眞井 翔子, 柳 輝希, 島野 麻由奈, 西江 渉, 氏家 英之, 有田 賢, 日本皮膚科学会雑誌, 131, 3, 543, 543, Mar. 2021
    (公社)日本皮膚科学会, Japanese
  • Serpiginous erythema on the trunk
    Takamasa Ito, Hideyuki Ujiie, The BMJ, 372, 21 Jan. 2021
    Report scientific journal
  • Childhood asymmetric labium majus enlargement(CALME)の1例               
    川村 拓也, 織田 晃央, 氏家 韻欣, 氏家 英之, 日本皮膚科学会雑誌, 131, 1, 128, 128, Jan. 2021
    (公社)日本皮膚科学会, Japanese
  • 類天疱瘡update               
    氏家 英之, 日本皮膚科学会雑誌, 131, 1, 121, 122, Jan. 2021
    (公社)日本皮膚科学会, Japanese
  • 原発不明癌を合併した抗ラミニン332型粘膜類天疱瘡の1例               
    葭本 倫大, 織田 晃央, 宮本 航大, 須貝 達朗, 藤村 悠, 氏家 韻欣, 夏賀 健, 清水 宏, 氏家 英之, 日本皮膚科学会雑誌, 130, 12, 2579, 2580, Nov. 2020
    (公社)日本皮膚科学会, Japanese
  • Familial chilblain lupus with TREX1 mutation and cerebrovascular disease
    Takuma Nohara, Teruki Yanagi, Ichiro Yabe, Nakao Ota, Nobuo Kanazawa, Hideyuki Ujiie, Hideyuki Kosumi, Yosuke Mai, Hiroshi Shimizu, LANCET RHEUMATOLOGY, 2, 11, E724, E724, Nov. 2020, [International Magazine]
    English
  • 治療法の再整理とアップデートのために 専門家による 私の治療 類天疱瘡
    氏家 英之, 日本医事新報, 5018, 43, 43, Jun. 2020
    (株)日本医事新報社, Japanese
  • DPP-4阻害薬服用中の糖尿病患者に生じた水疱性類天疱瘡               
    氏家 英之, 岩田 浩明, 葭本 倫大, 氏家 韻欣, 中村 昭伸, 三好 秀明, 清水 宏, 糖尿病, 63, 5, 355, 355, May 2020
    (一社)日本糖尿病学会, Japanese
  • 【検査値を読む2020】(19章)自己免疫検査 抗デスモグレイン抗体
    氏家 英之, 内科, 125, 4, 850, 850, Apr. 2020
    (株)南江堂, Japanese
  • 【検査値を読む2020】(19章)自己免疫検査 抗BP180NC16a抗体
    氏家 英之, 内科, 125, 4, 851, 851, Apr. 2020
    (株)南江堂, Japanese
  • TREX1遺伝子変異を認めた家族性凍瘡状ループス(Familial chilblain lupus:FCL)の2家系               
    野原 拓馬, 柳 輝希, 小住 英之, 眞井 洋輔, 宮澤 元, 佐藤 一正, 渡邉 美佳, 泉 健太郎, 氏家 英之, 清水 宏, 矢部 一郎, 太田 仲郎, 金澤 伸雄, 日本皮膚科学会雑誌, 130, 3, 393, 393, Mar. 2020
    (公社)日本皮膚科学会, Japanese
  • 粘膜類天疱瘡抗体によるBP180と4型コラーゲンの結合阻害               
    岩田 浩明, 鎌口 真由美, 西江 渉, 氏家 英之, 夏賀 健, 清水 宏, 日本皮膚科学会雑誌, 130, 3, 412, 412, Mar. 2020
    (公社)日本皮膚科学会, Japanese
  • 【水疱をどう診る?どう治す?】水疱性類天疱瘡と粘膜類天疱瘡
    氏家 英之, Derma., 292, 29, 37, Feb. 2020
    (株)全日本病院出版会, Japanese
  • 粘膜類天疱瘡の鑑別における蛍光抗体直接法の施行例のまとめ               
    岩田 浩明, 氏家 英之, 氏家 韻欣, 清水 宏, 日本皮膚科学会雑誌, 130, 1, 84, 84, Jan. 2020
    (公社)日本皮膚科学会, Japanese
  • 自己免疫性水疱症の診断の最前線
    氏家 英之, 検査と技術, 48, 1, 10, 13, Jan. 2020
    (株)医学書院, Japanese
  • 【日常診療で接する薬剤性皮膚障害】(Part1.)糖尿病治療薬による薬疹(総説1) DPP-4阻害薬内服に伴う水疱性類天疱瘡               
    氏家 英之, Visual Dermatology, 19, 2, 134, 139, Jan. 2020
    (株)学研メディカル秀潤社, Japanese
  • 【日常診療で接する薬剤性皮膚障害】(Part1.)糖尿病治療薬による薬疹(case1) DPP-4阻害薬内服患者に生じた水疱性類天疱瘡の1例               
    葭本 倫大, 氏家 英之, Visual Dermatology, 19, 2, 140, 142, Jan. 2020
    (株)学研メディカル秀潤社, Japanese
  • 眼粘膜症状を呈した後天性表皮水疱症の1例
    村田 真美, 浅野 伸幸, 氏家 英之, 山田 隆弘, 下村 裕, 西日本皮膚科, 81, 6, 478, 482, Dec. 2019
    日本皮膚科学会-西部支部, Japanese
  • Epitope spreading現象によって抗BP180 NC16a抗体が陽転化したdipeptidyl peptidase-IV阻害薬関連水疱性類天疱瘡の3例               
    眞井 洋輔, 西江 渉, 泉 健太郎, 葭本 倫大, 森田 裕介, 渡邉 美佳, 豊永 愛恋, 氏家 英之, 岩田 浩明, 藤田 靖幸, 乃村 俊史, 松村 和子, 清水 聡子, 清水 宏, 加齢皮膚医学セミナー, 14, 2, 67, 67, Dec. 2019
    加齢皮膚医学研究会, Japanese
  • 糖尿病をめぐる診療科リレー 皮膚科 糖尿病と皮膚疾患、特にDPP-4阻害薬と水疱性類天疱瘡の関連
    氏家 英之, DM Ensemble, 8, 3, 42, 45, Nov. 2019
    (公社)日本糖尿病協会, Japanese
  • 発症から診断までに10年以上が経過した粘膜類天疱瘡の1例
    鈴木 丈雄, 新熊 悟, 荻根沢 真帆子, 会沢 敦子, 高橋 奈央, 泉 健太郎, 氏家 英之, 西江 渉, 阿部 理一郎, 臨床皮膚科, 73, 10, 813, 817, Sep. 2019
    (株)医学書院, Japanese
  • CRISPR/Cas9システムを用いた劣性栄養障害型表皮水疱症(RDEB)の治療法の開発               
    高島 翔太, 乃村 俊史, 氏家 英之, 夏賀 健, 岩田 浩明, 中村 秀樹, 清水 宏, 新熊 悟, 阿部 理一郎, 藤田 靖幸, 日本皮膚科学会雑誌, 129, 9, 1909, 1909, Aug. 2019
    (公社)日本皮膚科学会, Japanese
  • 眼粘膜症状を呈した後天性表皮水疱症の1例
    村田 真美, 浅野 伸幸, 下村 裕, 氏家 英之, 西日本皮膚科, 81, 3, 235, 235, Jun. 2019
    日本皮膚科学会-西部支部, Japanese
  • Epitope spreading現象によって抗BP180 NC16a抗体が陽転化したdipeptidyl peptidase-IV阻害薬関連水疱性類天疱瘡の3例               
    眞井 洋輔, 西江 渉, 泉 健太郎, 葭本 倫大, 森田 裕介, 渡邉 美佳, 豊永 愛恋, 氏家 英之, 岩田 浩明, 藤田 靖幸, 乃村 俊史, 松村 和子, 清水 聡子, 清水 宏, 加齢皮膚医学セミナー, 14, 1, 65, 65, Jun. 2019
    加齢皮膚医学研究会, Japanese
  • 自己免疫性水疱症177例における治療前スクリーニング検査結果の検討               
    氏家 韻欣, 氏家 英之, 岩田 浩明, 清水 宏, 日本皮膚科学会雑誌, 129, 5, 1164, 1164, May 2019
    (公社)日本皮膚科学会, Japanese
  • 特発性CD4陽性リンパ球減少症の患者に生じた播種性Mycobacterium intracellulare感染症               
    清水 聡子, 真屋 由佳, 高島 有香, 児玉 文宏, 横関 真由美, 冨澤 幸生, 村松 憲, 氏家 英之, 清水 宏, 守内 玲寧, 日本皮膚科学会雑誌, 129, 5, 1221, 1221, May 2019
    (公社)日本皮膚科学会, Japanese
  • 診断に苦慮した粘膜類天疱瘡(mucous membrane pemphigoid;MMP)の1例               
    鈴木 丈雄, 新熊 悟, 荻根沢 真帆子, 会沢 敦子, 阿部 理一郎, 高橋 奈央, 氏家 英之, 日本皮膚科学会雑誌, 129, 4, 551, 551, Apr. 2019
    (公社)日本皮膚科学会, Japanese
  • 初発部位とは異なる部位に皮疹が生じた限局性水疱性類天疱瘡(LBP)の2例               
    長谷川 瑛人, 新熊 悟, 勝見 達也, 阿部 理一郎, 伊藤 薫, 笠原 直美, 池滝 勝史, 氏家 英之, 日本皮膚科学会雑誌, 129, 4, 551, 551, Apr. 2019
    (公社)日本皮膚科学会, Japanese
  • 類天疱瘡の基礎と最近の話題               
    氏家 英之, 日本皮膚科学会雑誌, 129, 4, 571, 572, Apr. 2019
    (公社)日本皮膚科学会, Japanese
  • 透析患者の粘膜優位に生じた非イオン性ヨード造影剤による固定薬疹の3例               
    瀬尾 拓志, 岩田 浩明, 眞井 洋輔, 山口 泰之, 大口 由香, 氏家 英之, 清水 宏, 日本皮膚科学会雑誌, 129, 4, 586, 586, Apr. 2019
    (公社)日本皮膚科学会, Japanese
  • 水疱部に著しい好中球浸潤を認めた水疱性類天疱瘡と尋常性乾癬の合併例               
    稲村 衣美, 岩田 浩明, 水上 卓哉, 野原 拓馬, 中山 ちひろ, 村松 憲, 辻脇 真澄, 秦 洋郎, 氏家 英之, 清水 宏, 日本皮膚科学会雑誌, 129, 4, 588, 588, Apr. 2019
    (公社)日本皮膚科学会, Japanese
  • Linear immunoglobulin A/immunoglobulin G bullous dermatosis with autoantibodies to LAD-1 and laminin-γ1
    Y. Matsudate, K. Yamasaki, H. Ujiie, H. Iwata, Y. Kubo, Clinical and Experimental Dermatology, 44, 3, e44, e46, Apr. 2019
    Report scientific journal
  • Helicobacter pylori除菌療法後に生じた皮疹の機序               
    伊東 孝政, 清水 宏, 白水 崇, 氏家 英之, 阿部 理一郎, 日本皮膚科学会雑誌, 129, 1, 45, 45, Jan. 2019
    (公社)日本皮膚科学会, Japanese
  • 尋常性乾癬に合併した組織学的に好中球浸潤が著しい類天疱瘡の1例               
    稲村 衣美, 岩田 浩明, 野原 拓馬, 水上 卓哉, 羽賀 直哉, 中山 ちひろ, 村松 憲, 辻脇 真澄, 秦 洋郎, 氏家 英之, 清水 宏, 日本皮膚科学会雑誌, 129, 1, 47, 47, Jan. 2019
    (公社)日本皮膚科学会, Japanese
  • 稗粒腫形成を認めたdipeptidyl peptidase-IV阻害薬関連水疱性類天疱瘡の4例               
    眞井 洋輔, 西江 渉, 氏家 英之, 泉 健太郎, 岩田 浩明, 秦 洋郎, 清水 宏, 水野 修, 伊藤 圭, 日本皮膚科学会雑誌, 128, 12, 2665, 2665, Nov. 2018
    (公社)日本皮膚科学会, Japanese
  • 重篤な喉頭粘膜疹により気管切開を要した水疱性類天疱瘡の1例
    鬼頭 由紀子, 浦野 聖子, 望月 大極, 関 敦郎, 氏家 英之, 西江 渉, 皮膚科の臨床, 60, 11, 1757, 1761, Oct. 2018
    金原出版(株), Japanese
  • A case of subepidermal autoimmune bullous disease with autoantibodies against 200 and 290-kDa antigens
    M. Sawada, T. Hida, H. Ujiie, H. Iwata, H. Uhara, Journal of the European Academy of Dermatology and Venereology, 32, 9, e354, e355, Sep. 2018
    Report scientific journal
  • Bullous pemphigoid with the deposition of IgG2 but not IgG1, IgG3 nor IgG4 autoantibodies at the basement membrane zone
    N. Yoshimoto, H. Ujiie, M. Zheng, H. Iwata, H. Kosumi, H. Hata, H. Shimizu, Journal of the European Academy of Dermatology and Venereology, 32, 9, e344, e346, Sep. 2018
    Report scientific journal
  • 【妊娠と皮膚】(Part2)妊娠に伴って出現、増悪する皮膚疾患(case 2) 線状IgA水疱性皮膚症               
    氏家 英之, 林 昌浩, Visual Dermatology, 17, 10, 936, 938, Sep. 2018
    (株)学研メディカル秀潤社, Japanese
  • 眼粘膜症状を呈した後天性表皮水疱症の1例               
    村田 真美, 浅野 伸幸, 下村 裕, 氏家 英之, 日本皮膚科学会雑誌, 128, 10, 2122, 2122, Sep. 2018
    (公社)日本皮膚科学会, Japanese
  • DPP-4阻害薬による類天疱瘡のリスク因子
    氏家 英之, 内分泌・糖尿病・代謝内科, 47, 3, 210, 214, Sep. 2018
    (有)科学評論社, Japanese
  • Epitope spreading現象によって抗BP180NC16a抗体が陽性となったdipeptidyl peptidase-IV阻害薬関連水疱性類天疱瘡の3例               
    眞井 洋輔, 西江 渉, 泉 健太郎, 葭本 倫大, 森田 裕介, 渡邉 美佳, 豊永 愛恋, 氏家 英之, 岩田 浩明, 藤田 靖幸, 乃村 俊史, 清水 宏, 松村 和子, 清水 聡子, 日本皮膚科学会雑誌, 128, 8, 1663, 1663, Jul. 2018
    (公社)日本皮膚科学会, Japanese
  • エリテマトーデスに対するヒドロキシクロロキンの使い方 CLEとSLEで投与方法などに違いはあるが、注意点は同様となる
    谷川 瑛子, 氏家 英之, 日本医事新報, 4913, 57, 58, Jun. 2018
    (株)日本医事新報社, Japanese
  • 抗デスモグレイン3抗体がCLEIAで陰性、ELISAで陽性となった尋常性天疱瘡の1例               
    眞井 洋輔, 氏家 英之, 西村 真智子, 古賀 浩嗣, 真屋 由佳, 得地 景子, 藤田 靖幸, 三河 洋平, 清水 宏, 西日本皮膚科, 80, 3, 289, 290, Jun. 2018
    日本皮膚科学会-西部支部, Japanese
  • A possible association between BP230-type bullous pemphigoid and dementia: a report of two cases in elderly patients
    M. Zheng, H. Ujiie, K. Muramatsu, K. C. Sato-Matsumura, T. Maeda, I. Ujiie, H. Iwata, K. Izumi, W. Nishie, H. Shimizu, British Journal of Dermatology, 178, 6, 1449, 1450, 01 Jun. 2018
    Blackwell Publishing Ltd, English, Report scientific journal
  • 経過中に免疫再構築症候群として粘膜症状が悪化したDPP4阻害剤関連自己免疫性水疱症の2例               
    杉山 聖子, 片山 智恵子, 陶守 真貴子, 泉 健太郎, 氏家 英之, 西江 渉, 妹尾 明美, 青山 裕美, 日本皮膚科学会雑誌, 128, 5, 1201, 1201, May 2018
    (公社)日本皮膚科学会, Japanese
  • CLEIAで検出できない抗デスモグレイン3抗体の特徴               
    眞井 洋輔, 氏家 英之, 東 恒仁, 岩田 浩明, 清水 宏, 日本皮膚科学会雑誌, 128, 5, 1201, 1201, May 2018
    (公社)日本皮膚科学会, Japanese
  • CLEIAで検出されない抗デスモグレイン3抗体は抗原との結合速度が遅い               
    眞井 洋輔, 氏家 英之, 東 恒仁, 岩田 浩明, 清水 宏, 日本皮膚科学会雑誌, 128, 4, 607, 607, Apr. 2018
    (公社)日本皮膚科学会, Japanese
  • 【最近のトピックス2018】皮膚疾患治療のポイント 類天疱瘡診療ガイドラインについて
    氏家 英之, 臨床皮膚科, 72, 5, 121, 124, Apr. 2018
    (株)医学書院, Japanese
  • 17型コラーゲンは加齢による表皮の過増殖を抑制する               
    渡邉 美佳, 夏賀 健, 西江 渉, 藤村 悠, 氏家 英之, 尾崎 倫孝, 清水 宏, 日本皮膚科学会雑誌, 128, 4, 607, 607, Apr. 2018
    (公社)日本皮膚科学会, Japanese
  • DPP-4阻害薬による類天疱瘡の発症 通常の薬疹とは異なる発症機序が推定される。HLA-DQB1*03:01がリスク因子
    氏家 英之, 大塚 篤司, 日本医事新報, 4901, 56, 56, Mar. 2018
    (株)日本医事新報社, Japanese
  • CLEIAで抗デスモグレイン3抗体が陰性となった尋常性天疱瘡の1例               
    眞井 洋輔, 氏家 英之, 西村 真智子, 真屋 由佳, 得地 景子, 藤田 靖幸, 岩田 浩明, 清水 宏, 三河 洋平, 古賀 浩嗣, 日本皮膚科学会雑誌, 128, 1, 61, 61, Jan. 2018
    (公社)日本皮膚科学会, Japanese
  • 【内科医が知っておくべき皮膚疾患】DPP-4阻害薬と類天疱瘡
    氏家 英之, Mebio, 34, 10, 92, 98, Oct. 2017
    (株)メジカルビュー社, Japanese
  • 乳癌を合併した後天性表皮水疱症の1例               
    緋田 哲也, 廣瀬 憲志, 村尾 和俊, 武知 浩和, 氏家 英之, 久保 宜明, 西日本皮膚科, 79, 5, 514, 514, Oct. 2017
    日本皮膚科学会-西部支部, Japanese
  • Type XVII collagen suppresses interfollicular epidermal proliferation in neonatal and aged skin, and helps rejuvenate epidermis
    M. Watanabe, K. Natsuga, W. Nishie, G. Donati, Y. Fujimura, T. Tsukiyama, H. Ujiie, M. Ozaki, F. M. Watt, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 10, S207, S207, Oct. 2017
    English, Summary international conference
  • Treg dysfunction leads to spontaneous production of autoantibodies to bullous pemphigoid antigens in mice
    K. Muramatsu, H. Ujiie, W. Nishie, K. Izumi, T. Ito, H. Iwata, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 10, S254, S254, Oct. 2017
    English, Summary international conference
  • High-dose immunoglobulin contains anti-idiotypic antibodies against BP-IgG
    M. Kamaguchi, H. Iwata, Y. Mori, H. Ujiie, Y. Kitagawa, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 10, S243, S243, Oct. 2017
    English, Summary international conference
  • IgA型ALアミロイドーシスに線状IgA皮膚症を併発した1例               
    山口 泰之, 氏家 英之, 大東 寛幸, 岩田 浩明, 村松 憲, 清水 宏, 遠藤 知之, 豊嶋 崇徳, 日本皮膚科学会雑誌, 127, 9, 2111, 2111, Aug. 2017
    (公社)日本皮膚科学会, Japanese
  • Juvenile spring eruptionの1例               
    白戸 貴久, 西村 真智子, 新熊 悟, 氏家 英之, 乃村 俊史, 藤田 靖幸, 清水 宏, 阿部 理一郎, 日本皮膚科学会雑誌, 127, 9, 2114, 2114, Aug. 2017
    (公社)日本皮膚科学会, Japanese
  • Dipeptidyl peptidase-IV阻害薬(DPP4i)関連水疱性類天疱瘡の臨床・免疫学的検討               
    泉 健太郎, 西江 渉, 眞井 洋輔, 氏家 英之, 岩田 浩明, 清水 宏, 日本皮膚科学会雑誌, 127, 9, 2118, 2118, Aug. 2017
    (公社)日本皮膚科学会, Japanese
  • IgA型ALアミロイドーシスに線状IgA皮膚症を併発した1例               
    山口 泰之, 氏家 英之, 大東 寛幸, 岩田 浩明, 村松 憲, 清水 宏, 遠藤 知之, 豊嶋 崇徳, 日本皮膚科学会雑誌, 127, 9, 2111, 2111, Aug. 2017
    (公社)日本皮膚科学会, Japanese
  • 皮膚粘膜移行部と手掌に限局した抗ラミニンγ1類天疱瘡               
    大日 輝記, 椛島 健治, 本田 由貴, 服部 ゆかり, 宮地 良樹, 西江 渉, 氏家 英之, 日本皮膚科学会雑誌, 127, 7, 1562, 1562, Jun. 2017
    (公社)日本皮膚科学会, Japanese
  • 【診断基準を満たさないとき、どうする?】(Part2)非典型例をどう考えるか(case8) 類天疱瘡               
    氏家 英之, Visual Dermatology, 16, 7, 670, 674, Jun. 2017
    (株)学研メディカル秀潤社, Japanese
  • 日本皮膚科学会ガイドライン 類天疱瘡(後天性表皮水疱症を含む)診療ガイドライン
    氏家 英之, 岩田 浩明, 山上 淳, 名嘉眞 武国, 青山 裕美, 池田 志斈, 石井 文人, 岩月 啓氏, 黒沢 美智子, 澤村 大輔, 谷川 瑛子, 鶴田 大輔, 西江 渉, 藤本 亘, 天谷 雅行, 清水 宏, 類天疱瘡(後天性表皮水疱症を含む)診療ガイドライン作成委員会, 日本皮膚科学会雑誌, 127, 7, 1483, 1521, Jun. 2017
    (公社)日本皮膚科学会, Japanese
  • 自己免疫性水疱症の病態と治療に関する新しい話題 DPP-4阻害薬内服に伴う水疱性類天疱瘡               
    氏家 英之, 日本皮膚科学会雑誌, 127, 5, 977, 977, May 2017
    (公社)日本皮膚科学会, Japanese
  • マウス抗原を標的とした水疱性類天疱瘡マウスモデルの作製               
    氏家 英之, 日本皮膚科学会雑誌, 127, 5, 1081, 1081, May 2017
    (公社)日本皮膚科学会, Japanese
  • ラミニンγ1に対する自己抗体も有した線状IgA/IgG水疱性皮膚症の1例               
    松立 吉弘, 山崎 佳那子, 矢田 未央, 村尾 和俊, 久保 宜明, 佐川 禎昭, 氏家 英之, 岩田 浩明, 日本皮膚科学会雑誌, 127, 5, 1159, 1159, May 2017
    (公社)日本皮膚科学会, Japanese
  • 【押さえておきたい新しい指定難病】類天疱瘡(後天性表皮水疱症を含む)(疾病番号162)
    氏家 英之, Derma., 257, 41, 47, May 2017
    (株)全日本病院出版会, Japanese
  • Gene-edition targeting mutation site recovers null-expression of type VII collagen caused by frameshift mutation via non-homologous end joining in Recessive Dystrophic Epidermolysis Bullosa
    S. Takashima, S. Shinkukma, Y. Fujita, T. Nomura, H. Ujiie, R. Abe, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 137, 5, S83, S83, May 2017
    English, Summary international conference
  • ENPP1に新規ミスセンス変異を同定したCole病の1家系               
    宮内 俊成, 乃村 俊史, 鈴木 翔多朗, 竹田 真依, 新熊 悟, 氏家 英之, 藤田 靖幸, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 127, 5, 1170, 1170, May 2017
    (公社)日本皮膚科学会, Japanese
  • Bullous pemphigoid developed in a patient with prurigo nodularis
    N. Yoshimoto, H. Ujiie, Y. Hirata, K. Izumi, W. Nishie, H. Shimizu, JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY, 31, 4, E187, E189, Apr. 2017
    English, Report scientific journal
  • 色素異常を伴う掌蹠角化症の1家系               
    宮内 俊成, 乃村 俊史, 鈴木 翔多朗, 竹田 真依, 新熊 悟, 氏家 英之, 藤田 靖幸, 清水 宏, 阿部 理一郎, 角化症研究会記録集, 31, 114, 117, Mar. 2017
    角化症研究会事務局, Japanese
  • Linagliptin-associated bullous pemphigoid that was most likely caused by IgG autoantibodies against the midportion of BP180
    A. Sakai, Y. Shimomura, O. Ansai, Y. Saito, K. Tomii, Y. Tsuchida, H. Iwata, H. Ujiie, H. Shimizu, R. Abe, British Journal of Dermatology, 176, 2, 541, 543, 01 Feb. 2017
    Blackwell Publishing Ltd, English, Report scientific journal
  • IgE autoantibodies in bullous pemphigoid
    H. Ujiie, British Journal of Dermatology, 177, 6, 1481, 1482, 2017
    Blackwell Publishing Ltd, English, Report scientific journal
  • IgEが関与する皮膚疾患 アトピー性皮膚炎と水疱性類天疱瘡の病態
    氏家 英之, 北海道医学雑誌, 91, 2, 99, 99, Nov. 2016
    北海道医学会, Japanese
  • Subepidermal autoimmune bullous disease affecting predominantly mucocutaneous junctions and the palms with autoantibodies to BP230 and laminin γ1
    Y. Honda, T. Dainichi, W. Nishie, H. Ujiie, Y. Hattori, Y. Miyachi, K. Kabashima, British Journal of Dermatology, 175, 3, 619, 621, 01 Sep. 2016
    Blackwell Publishing Ltd, English, Report scientific journal
  • Macropinocytosis of type XVII collagen induced by bullous pemphigoid IgG is regulated by protein kinase C
    H. Iwata, H. Ujiie, K. Izumi, K. Natsuga, S. Shinkuma, W. Nishie, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 9, S172, S172, Sep. 2016
    English, Summary international conference
  • Not only ectodomain shedding but also C-terminal cleavage induces neoepitopes on proteolyzed collagen XVII
    E. Toyonaga, W. Nishie, K. Izumi, H. Ujiie, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 9, S178, S178, Sep. 2016
    English, Summary international conference
  • A novel mechanism of skin reaction related to Helicobacter pylori treatment
    T. Ito, H. Ujiie, Y. Fujita, H. Shimizu, R. Abe, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 9, S165, S165, Sep. 2016
    English, Summary international conference
  • Collagen XVII is the major autoantigen in mucous membrane pemphigoid
    K. Izumi, W. Nishie, M. Nishimura, H. Ujiie, H. Iwata, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 9, S213, S213, Sep. 2016
    English, Summary international conference
  • 【リンフォーマ・白血病】 顔面の巨大な腫瘤に対して電子線照射療法が著効した菌状息肉症の1例
    葭本 倫大, 藤田 靖幸, 平田 悠, 保科 大地, 守内 玲寧, 氏家 英之, 井上 哲也, 清水 宏, 皮膚科の臨床, 58, 10, 1483, 1487, Sep. 2016
    金原出版(株), Japanese
  • 【肥満細胞は今】自己免疫性水疱症と肥満細胞
    氏家 英之, 皮膚アレルギーフロンティア, 14, 2, 77, 80, Jul. 2016
    (株)メディカルレビュー社, Japanese
  • 市販薬内服後に発症しラミニンα3に対する自己抗体が陽性であった表皮下水疱症               
    菊地 一博, 伊藤 圭, 羽賀 直哉, 水野 修, 氏家 英之, 村松 憲, 荻野 次郎, 田中 祥恵, 村本 文男, 日本皮膚科学会雑誌, 126, 7, 1320, 1320, Jun. 2016
    (公社)日本皮膚科学会, Japanese
  • 顔面の腫瘤に対し電子線照射およびRePUVA療法が著効した腫瘤期菌状息肉症の1例               
    葭本 倫大, 藤田 靖幸, 平田 悠, 保科 大地, 守内 玲寧, 氏家 英之, 清水 宏, 井上 哲也, 日本皮膚科学会雑誌, 126, 7, 1331, 1331, Jun. 2016
    (公社)日本皮膚科学会, Japanese
  • 類天疱瘡の診療ガイドライン(案)から推奨される治療戦略               
    氏家 英之, 日本皮膚科学会雑誌, 126, 5, 731, 731, May 2016
    (公社)日本皮膚科学会, Japanese
  • Complement-independent blister formation in IgG4-dominant bullous pemphigoid
    T. Dainichi, W. Nishie, Y. Yamagami, H. Sonobe, H. Ujiie, Y. Kaku, K. Kabashima, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 136, 5, S7, S7, May 2016
    English, Summary international conference
  • 顔面の巨大な腫瘤に対し電子線照射およびRe-PUVA療法が著効した菌状息肉症の1例               
    葭本 倫大, 藤田 靖幸, 平田 悠, 保科 大地, 守内 玲寧, 氏家 英之, 清水 宏, 井上 哲也, 日本皮膚科学会雑誌, 126, 2, 167, 167, Feb. 2016
    (公社)日本皮膚科学会, Japanese
  • 水疱性類天疱瘡におけるIgE自己抗体の解析
    守内 玲寧, 西江 渉, 氏家 英之, 夏賀 健, 清水 宏, 日本皮膚科学会雑誌, 125, 12, 2301, 2301, Nov. 2015
    (公社)日本皮膚科学会, Japanese
  • Mycosis fungoides associated with splenic infarction and muscle involvement
    M. Watanabe, H. Ujiie, K. Nishimura, T. Kamiyama, R. Abe, H. Shimizu, BRITISH JOURNAL OF DERMATOLOGY, 173, 4, 1100, 1102, Oct. 2015
    English, Report scientific journal
  • Autoantibodies to parts of type XVII collagen outside of the non-collageneous 16A domain lead to mild bullous pemphigoid due to the non-depletion of autoantigen
    H. Iwata, K. Imafuku, K. Izumi, M. Wada, K. Natsuga, H. Ujiie, W. Nishie, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 135, S4, S4, Sep. 2015
    English, Summary international conference
  • 抗NC16A抗体を有さない軽症の水疱性類天疱瘡の1例               
    今福 恵輔, 岩田 浩明, 泉 健太郎, 夏賀 健, 氏家 英之, 西江 渉, 清水 宏, 日本皮膚科学会雑誌, 125, 4, 915, 915, Apr. 2015
    (公社)日本皮膚科学会, Japanese
  • 水疱性類天疱瘡自己抗原である17型コラーゲンの異なるエピトープに対する抗体の病原性解析               
    和田 麻友美, 西江 渉, 氏家 英之, 泉 健太郎, 岩田 浩明, 夏賀 健, 清水 宏, 北川 善政, 北海道歯学雑誌, 35, 2, 188, 188, Mar. 2015
    北海道歯学会, Japanese
  • ヒト17型コラーゲン全長リコンビナントタンパクを用いた新規ELISAの確立               
    泉 健太郎, 西江 渉, 氏家 英之, 清水 宏, 日本皮膚科学会雑誌, 124, 10, 1928, 1929, Sep. 2014
    (公社)日本皮膚科学会, Japanese
  • gamma delta T cells protect TCR alpha deficient mice from the autoimmunity induced by scurfy lymphocytes
    Hideyuki Ujiie, Ethan Shevach, JOURNAL OF IMMUNOLOGY, 192, May 2014
    English, Summary international conference
  • Alternaria alternata感染による深在性皮膚真菌症の1例
    柴 景子, 大口 由香, 青柳 哲, 氏家 英之, 西谷 道子, 鎗田 響子, 亀井 克彦, 清水 宏, 臨床皮膚科, 68, 4, 355, 359, Apr. 2014
    (株)医学書院, Japanese
  • 成人型肥満細胞症の2例
    中里 信一, 藤田 靖幸, 氏家 英之, 西江 渉, 阿部 理一郎, 近藤 健, 清水 宏, 皮膚科の臨床, 56, 4, 595, 599, Apr. 2014
    金原出版(株), Japanese
  • 著明な粘液を伴ったグロムス腫瘍の1例
    柴 景子, 本間 英里奈, 青柳 哲, 堀江 啓太, 秦 洋郎, 大澤 倫子, 氏家 英之, 高橋 祥公, 清水 宏, 皮膚科の臨床, 56, 1, 111, 114, Jan. 2014
    金原出版(株), Japanese
  • Characteristic findings of handprint and dermoscopy in reticulate acropigmentation of Kitamura
    H. Koguchi, H. Ujiie, S. Aoyagi, R. Osawa, H. Shimizu, CLINICAL AND EXPERIMENTAL DERMATOLOGY, 39, 1, 85, 87, Jan. 2014
    English, Report scientific journal
  • 著明な粘液を伴ったグロムス腫瘍の1例               
    柴 景子, 本間 英里奈, 青柳 哲, 堀江 啓太, 大澤 倫子, 氏家 英之, 秦 洋郎, 清水 宏, 高橋 祥公, 日本皮膚科学会雑誌, 123, 8, 1544, 1544, Jul. 2013
    (公社)日本皮膚科学会, Japanese
  • 成人型肥満細胞症の2例               
    中里 信一, 藤田 靖幸, 氏家 英之, 西江 渉, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 123, 8, 1545, 1545, Jul. 2013
    (公社)日本皮膚科学会, Japanese
  • 深在性皮膚アルテルナリア症の1例               
    柴 景子, 大口 由香, 青柳 哲, 氏家 英之, 清水 宏, 西谷 道子, 鎗田 響子, 亀井 克彦, 日本皮膚科学会雑誌, 123, 8, 1545, 1545, Jul. 2013
    (公社)日本皮膚科学会, Japanese
  • 悪性黒色腫との鑑別を要した左中指の色素性Bowen病               
    山口 真規子, 本間 英里奈, 青柳 哲, 柴 景子, 中里 信一, 大宮 若菜, 秦 洋郎, 氏家 英之, 清水 宏, 日本皮膚科学会雑誌, 123, 8, 1549, 1549, Jul. 2013
    (公社)日本皮膚科学会, Japanese
  • 左前腕の紅色結節               
    藤田 靖幸, 青柳 哲, 辻脇 真澄, 本間 英里奈, 氏家 英之, 藤田 裕美, 畑中 佳奈子, 清水 宏, 日本皮膚病理組織学会抄録集, 29回, 15, 15, Jul. 2013
    日本皮膚病理組織学会, Japanese
  • 水疱性類天疱瘡と落葉状天疱瘡が合併した1例               
    辻脇 真澄, 阿部 理一郎, 野村 友希子, 保科 大地, 山根 尚子, 新熊 悟, 氏家 英之, 有田 賢, 清水 宏, 日本皮膚科学会雑誌, 123, 8, 1543, 1543, Jul. 2013
    (公社)日本皮膚科学会, Japanese
  • 著明な粘液を伴ったグロムス腫瘍の1例               
    柴 景子, 本間 英里奈, 青柳 哲, 堀江 啓太, 秦 洋郎, 大澤 倫子, 氏家 英之, 高橋 祥公, 日本皮膚科学会雑誌, 123, 6, 1078, 1078, May 2013
    (公社)日本皮膚科学会, Japanese
  • Bullous pemphigoid autoantibodies induce skin fragility in neonatal mice in a complement independent manner
    T. Sasaoka, H. Ujiie, W. Nishie, A. Shibaki, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S29, S29, May 2013
    English, Summary international conference
  • Detection of pathological cleavage sites and neoepitopes on collagen XVII, a major autoantigen for bullous pemphigoid
    W. Nishie, H. Ujiie, K. Izumi, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S20, S20, May 2013
    English, Summary international conference
  • Establishment of novel ELISA using full-length recombinant human collagen XVII
    K. Izumi, W. Nishie, H. Ujiie, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S33, S33, May 2013
    English, Summary international conference
  • Human type VII collagen harbors multiple pathogenically relevant epitopes
    A. Vorobyev, H. Ujiie, A. Recke, J. A. Buijsrogge, M. F. Jonkman, T. Hashimoto, S. C. Kim, J. H. Kim, R. Groves, E. Schmidt, D. Zillikens, H. Shimizu, R. J. Ludwig, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S45, S45, May 2013
    English, Summary international conference
  • Recombinant anti-collagen VII Fab fragments which compete against autoantibodies in patients with epidermolysis bullosa acquisita
    H. Qiao, H. Ujiie, R. Abe, Q. Li, R. Moriuchi, J. R. McMillan, H. Shimizu, G. Wang, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 133, S26, S26, May 2013
    English, Summary international conference
  • 水疱性類天疱瘡と落葉状天疱瘡の合併例-表皮間・基底膜自己抗体におけるIgG subclassの検討               
    辻脇 真澄, 野村 友希子, 保科 大地, 山根 尚子, 新熊 悟, 氏家 英之, 清水 宏, 有田 賢, 日本皮膚科学会雑誌, 123, 6, 1084, 1084, May 2013
    (公社)日本皮膚科学会, Japanese
  • Alternaria alternata感染による深在性皮膚真菌症の1例               
    大口 由香, 青柳 哲, 柴 景子, 氏家 英之, 西谷 道子, 鎗田 響子, 亀井 克彦, 清水 宏, 日本皮膚科学会雑誌, 123, 5, 989, 989, Apr. 2013
    (公社)日本皮膚科学会, Japanese
  • ケラチン5のコイル1A領域N末端部にミスセンス変異を生じた単純型表皮水疱症               
    新熊 悟, 西江 渉, 夏賀 健, 氏家 英之, 中村 秀樹, 清水 宏, Jacyk Witold, 秋山 真志, 日本皮膚科学会雑誌, 123, 4, 461, 461, Apr. 2013
    (公社)日本皮膚科学会, Japanese
  • 右鎖骨上窩リンパ節腫脹を契機に発見された食道原発悪性黒色腫の1例
    堀江 啓太, 秦 洋郎, 馬場 慶子, 泉 健太郎, 本間 英里奈, 氏家 英之, 青柳 哲, 細川 正夫, 清水 宏, 臨床皮膚科, 67, 3, 249, 253, Mar. 2013
    (株)医学書院, Japanese
  • Human type VII collagen harbors multiple pathogenically relevant epitopes
    A. Vorobyev, H. Ujiie, A. Recke, J. Buijsrogge, M. Jonkman, T. Hashimoto, S. Kim, J. Kim, R. Groves, E. Schmidt, D. Zillikens, H. Shimizu, R. J. Ludwig, EXPERIMENTAL DERMATOLOGY, 22, 3, E33, E33, Mar. 2013
    English, Summary international conference
  • Antibodies to pathogenic epitopes on type X VII collagen cause skin fragility in a complement-dependent and -independent manner
    夏賀 健, 西江 渉, 新熊 悟, 氏家 英之, 西村 真知子, 澤村 大輔, 清水 宏, 北海道醫學雜誌 = Acta medica Hokkaidonensia, 87, 6, 262, 262, 01 Nov. 2012
    Japanese
  • 右鎖骨上窩リンパ節腫張を契機に発見された食道原発悪性黒色腫の1例               
    堀江 啓太, 秦 洋郎, 馬場 慶子, 泉 健太郎, 本間 英里奈, 氏家 英之, 青柳 哲, 清水 宏, 細川 正夫, 日本皮膚科学会雑誌, 122, 10, 2506, 2506, Sep. 2012
    (公社)日本皮膚科学会, Japanese
  • Neoepitopes on the modified amino-terminus of the shed ectodomain of collagen XVII
    W. Nishie, H. Ujiie, S. Shinkuma, L. Bruckner-Tuderman, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 132, S22, S22, Sep. 2012
    English, Summary international conference
  • Generation of recombinant anti-collagen VII Fab fragments which compete against autoantibodies in patients with epidermolysis bullosa acquisita
    Qiao Hong-jiang, Hideyuki Ujiie, Riichiro Abe, Li Qiang, Reine Moriuchi, James R. McMillan, Wang Gang, Hiroshi Shimizu, JOURNAL OF DERMATOLOGY, 39, 29, 29, Jun. 2012
    English, Summary international conference
  • Intraepidermal neutrophilic IgA pemphigus successfully treated with dapson (vol 22, pg 282, 2012)
    Yu Hirata, Riichiro Abe, Kazuhiro Kikuchi, Asuka Hamasaka, Satoru Shinkuma, Hideyuki Ujiie, Toshifumi Nomura, Wataru Nishie, Ken Arita, Hiroshi Shimizu, EUROPEAN JOURNAL OF DERMATOLOGY, 22, 3, 429, 429, May 2012
    English, Others
  • 右鎖骨上窩リンパ節腫張を契機に発見された食道原発悪性黒色腫の1例               
    堀江 啓太, 秦 洋郎, 馬場 慶子, 泉 健太郎, 本間 英里奈, 氏家 英之, 青柳 哲, 細川 正夫, 清水 宏, 日本皮膚科学会雑誌, 122, 4, 1236, 1236, Apr. 2012
    (公社)日本皮膚科学会, Japanese
  • 新規水疱性類天疱瘡アクティブマウスモデルにおけるCD4陽性T細胞サブセットの役割の解析               
    氏家 英之, 西江 渉, 清水 宏, 日本皮膚科学会雑誌, 122, 4, 1258, 1258, Apr. 2012
    (公社)日本皮膚科学会, Japanese
  • 抗BP230抗体陽性pemphigoid nodularisの1例               
    新熊 悟, 猪熊 大輔, 夏賀 健, 氏家 英之, 西江 渉, 清水 宏, 日本皮膚科学会雑誌, 122, 3, 625, 625, Mar. 2012
    (公社)日本皮膚科学会, Japanese
  • 重篤な眼症状急激な視力障害を伴った木村氏病の1例               
    渡邉 美佳, 氏家 英之, 齋藤 奈央, 柳 輝希, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 122, 2, 376, 376, Feb. 2012
    (公社)日本皮膚科学会, Japanese
  • 骨髄異形成症候群を合併した壊疽性膿皮症の1例               
    松村 若菜, 氏家 英之, 泉 健太郎, 秋山 真志, 清水 宏, 今野 哲, 重松 明男, 小野沢 真弘, 日本皮膚科学会雑誌, 122, 2, 377, 377, Feb. 2012
    (公社)日本皮膚科学会, Japanese
  • 新規水疱性類天疱瘡マウスモデルの樹立と解析               
    氏家 英之, 芝木 晃彦, 西江 渉, 澤村 大輔, 王 剛, 立石 八寿貴, 李 強, 守内 玲寧, 喬 洪江, 中村 秀樹, 秋山 真志, 清水 宏, 日本皮膚科学会雑誌, 122, 2, 379, 379, Feb. 2012
    (公社)日本皮膚科学会, Japanese
  • 造血幹細胞移植を行った菌状息肉症・セザリー症候群の5例               
    渡邉 美佳, 氏家 英之, 山根 尚子, 阿部 理一郎, 清水 宏, 西尾 充史, 日本皮膚科学会雑誌, 122, 2, 382, 382, Feb. 2012
    (公社)日本皮膚科学会, Japanese
  • 日本人に生じたDuhring疱疹状皮膚炎における表皮トランスグルタミナーゼ(eTG)抗体価の検討               
    新熊 悟, 阿部 理一郎, 林 欣宇, 夏賀 健, 氏家 英之, 西江 渉, 清水 宏, 大田 光仁, 土屋 喜久夫, 福本 隆也, 浅田 秀夫, 日本皮膚科学会雑誌, 122, 2, 383, 383, Feb. 2012
    (公社)日本皮膚科学会, Japanese
  • 筋肉内浸潤および脾梗塞を呈した進行期菌状息肉症の1例               
    渡邉 美佳, 氏家 英之, 馬場 慶子, 芝木 晃彦, 阿部 理一郎, 清水 宏, 山田 洋介, 山口 圭介, 神山 俊哉, 日本皮膚科学会雑誌, 122, 1, 64, 64, Jan. 2012
    (公社)日本皮膚科学会, Japanese
  • 皮膚結節性アミロイドーシスの2例               
    泉 健太郎, 猪熊 大輔, 笠井 麻希, 藤田 靖幸, 氏家 英之, 山中 快子, 伊藤 圭, 清水 宏, 日本皮膚科学会雑誌, 122, 1, 51, 51, Jan. 2012
    (公社)日本皮膚科学会, Japanese
  • 塊状のIgA沈着を呈したDuhring疱疹状皮膚炎の1例               
    林 欣宇, 氏家 英之, 渡邉 美佳, 馬場 慶子, 新熊 悟, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 122, 1, 64, 64, Jan. 2012
    (公社)日本皮膚科学会, Japanese
  • Mohs氏軟膏療法を試みた転移性皮膚悪性腫瘍の3例               
    渡邊 英里香, 加藤 直子, 村田 純子, 斎藤 奈央, 氏家 英之, 田中 寛之, 日本皮膚科学会雑誌, 121, 14, 3361, 3361, Dec. 2011
    (公社)日本皮膚科学会, Japanese
  • 自己免疫性水疱症の最新知見 自己免疫性水疱症発症機序解明のための最新研究
    氏家 英之, 日本皮膚科学会雑誌, 121, 13, 2971, 2973, Dec. 2011
    (公社)日本皮膚科学会, Japanese
  • 水疱性類天疱瘡 病態と治療update               
    守内 玲寧, 氏家 英之, 西江 渉, 清水 宏, BIO Clinica, 26, 10, 957, 961, Sep. 2011
    (株)北隆館, Japanese
  • 自己免疫水疱症の最新知見 自己免疫性水疱症発生機序解明のための最新研究               
    氏家 英之, 日本皮膚科学会雑誌, 121, 3, 437, 437, Mar. 2011
    (公社)日本皮膚科学会, Japanese
  • タクロリムス軟膏外用が奏効した爪扁平苔癬               
    氏家 英之, 芝木 晃彦, 秋山 真志, 清水 宏, 皮膚病診療, 33, 3, 299, 302, Mar. 2011
    (株)協和企画, Japanese
  • 【自己免疫性水疱症 今日の進歩】水疱性類天疱瘡の病態解明 動物モデルを用いた最新研究
    氏家 英之, 医学のあゆみ, 236, 11, 1027, 1032, Mar. 2011
    医歯薬出版(株), Japanese
  • トラニラストの内服が有効であったeruptive syringomaの2例               
    堀江 啓太, 新熊 悟, 藤田 靖幸, 氏家 英之, 剱持 靖子, 青柳 哲, 清水 宏, 日本皮膚科学会雑誌, 121, 3, 605, 605, Mar. 2011
    (公社)日本皮膚科学会, Japanese
  • Psoriatic onycho-pachydermo periostitis that progressed to generalized pustular psoriasis
    Mika Watanabe, Hideyuki Ujiie, Maria Iitani Maroto, Riichiro Abe, Hiroshi Shimizu, JOURNAL OF DERMATOLOGY, 37, 120, 121, Sep. 2010
    English, Summary international conference
  • Erratum: Bone marrow transplantation restores epidermal basement membrane protein expression and rescues epidermolysis bullosa model mice (Proceedings of the National Academy of Sciences of the United States of America (2010) 107, (14345-14350) DOI: 10.10
    Yasuyuki Fujita, Riichiro Abe, Daisuke Inokuma, Mikako Sasaki, Daichi Hoshina, Ken Natsuga, Wataru Nishie, James R. McMillan, Hideki Nakamura, Tadamichi Shimizu, Masashi Akiyama, Daisuke Sawamura, Hiroshi Shimizu, Proceedings of the National Academy of Sciences of the United States of America, 107, 14514, 10 Aug. 2010
  • Mohs氏軟膏療法を試みた原発性および転移性皮膚悪性腫瘍の6例
    渡邊 英里香, 加藤 直子, 村田 純子, 斎藤 奈央, 氏家 英之, 田中 寛之, 臨床皮膚科, 64, 8, 605, 608, Jul. 2010
    (株)医学書院, Japanese
  • Human IgG1 mAb against human COL17 NC16A developed from BP patients induces blisters in experimental bullous pemphigoid model
    A. Shibaki, Q. Li, H. Ujiie, G. Wang, R. Moriuchi, H. Qiao, H. Morioka, S. Shinkuma, K. Natsuga, H. A. Long, W. Nishie, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 130, S11, S11, Apr. 2010
    English, Summary international conference
  • 筋肉内浸潤および脾梗塞を呈した進行期菌状息肉症の1例               
    渡邉 美佳, 氏家 英之, 馬場 慶子, 阿部 理一郎, 芝木 晃彦, 山田 洋介, 山口 圭介, 神山 俊哉, 清水 宏, 日本皮膚科学会雑誌, 120, 3, 743, 743, Mar. 2010
    (公社)日本皮膚科学会, Japanese
  • 塊状のIgA沈着を呈したDuhring疱疹状皮膚炎の1例               
    林 欣宇, 氏家 英之, 渡邉 美佳, 馬場 慶子, 新熊 悟, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 120, 3, 696, 696, Mar. 2010
    (公社)日本皮膚科学会, Japanese
  • 汎発性膿疱性乾癬に進展したPsoriatic onychopachydermo periostitis(POPP)の1例               
    渡邉 美佳, 氏家 英之, 飯谷 麻里, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 120, 1, 82, 82, Jan. 2010
    (公社)日本皮膚科学会, Japanese
  • タクロリムス軟膏外用が奏効した爪扁平苔癬5例のまとめ               
    氏家 英之, 芝木 晃彦, 秋山 真志, 清水 宏, 日本皮膚科学会雑誌, 120, 1, 82, 82, Jan. 2010
    (公社)日本皮膚科学会, Japanese
  • 汎発性膿疱性乾癬に進展したPsoriatic onychopachydermo periostitis(POPP)の1例               
    渡邉 美佳, 氏家 英之, 飯谷 麻里, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 120, 1, 87, 87, Jan. 2010
    (公社)日本皮膚科学会, Japanese
  • 皮膚結節性アミロイドーシスの2例               
    泉 健太郎, 猪熊 大輔, 笠井 麻希, 藤田 靖幸, 氏家 英之, 山中 快子, 伊藤 圭, 清水 宏, 日本皮膚科学会雑誌, 119, 4, 752, 752, Mar. 2009
    (公社)日本皮膚科学会, Japanese
  • 骨浸潤を伴ったエクリン汗孔癌の2例               
    山根 尚子, 加藤 直子, 柳 輝希, 大澤 倫子, 皆川 英彦, 合田 猛俊, 井須 和男, 氏家 英之, 日本皮膚科学会雑誌, 119, 1, 61, 61, Jan. 2009
    (公社)日本皮膚科学会, Japanese
  • 複数の皮膚悪性腫瘍に罹患し、ABCA12遺伝子の新規変異が同定された非水疱型先天性魚鱗癬様紅皮症の2例               
    夏賀 健, 秋山 真志, 坂井 香織, 中桐 頼子, 西村 真智子, 安倍 将隆, 有田 賢, 阿部 由紀子, 小野塚 貴, 青柳 哲, 秦 洋郎, 小玉 和郎, 清水 宏, 加藤 直子, 氏家 英之, 冨田 幸希, 日本皮膚科学会雑誌, 119, 1, 71, 71, Jan. 2009
    (公社)日本皮膚科学会, Japanese
  • Anaplastic large cell lymphoma(ALCL)の1例               
    山根 尚子, 加藤 直子, 柳 輝希, 大澤 倫子, 夏賀 健, 氏家 英之, 富田 幸希, 日本皮膚科学会雑誌, 119, 1, 73, 73, Jan. 2009
    (公社)日本皮膚科学会, Japanese
  • 【ケブネル現象をめぐって】比較的皮膚に限局した疾患 乾癬+扁平苔癬+尋常性白斑               
    氏家 英之, 澤村 大輔, 清水 宏, Visual Dermatology, 8, 1, 26, 27, Dec. 2008
    (株)学研メディカル秀潤社, Japanese
  • 【汗から探る皮膚病 汗は善玉か、悪玉か?】病変の深さによる整理 汗疱(IVIG治療中に生じたもの)               
    氏家 英之, 澤村 大輔, 清水 宏, Visual Dermatology, 7, 9, 974, 975, Aug. 2008
    (株)学研メディカル秀潤社, Japanese
  • DDSが奏効した深在性エリテマトーデスの1例               
    氏家 英之, 清水 忠道, 伊藤 幹, 有田 賢, 清水 宏, 日本皮膚科学会雑誌, 118, 7, 1271, 1271, Jun. 2008
    (公社)日本皮膚科学会, Japanese
  • 非水疱型先天性魚鱗癬様紅皮症(NBCIE)患者に生じた悪性黒色腫と末梢T細胞性リンパ腫(PTCL)               
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 日本皮膚科学会雑誌, 118, 7, 1278, 1279, Jun. 2008
    (公社)日本皮膚科学会, Japanese
  • 潰瘍を伴う体幹の硬結               
    小玉 和郎, 守内 玲寧, 氏家 英之, 有田 賢, 清水 忠道, 清水 宏, 南本 俊之, 日本皮膚科学会雑誌, 118, 6, 1108, 1108, May 2008
    (公社)日本皮膚科学会, Japanese
  • A Case of Bullous Pemphigoid Successfully Treated with Intravenous Immunoglobulin
    SHINKUMA Satoru, TSUJI ABE Yukiko, TOMITA Yuki, NATSUGA Ken, UJIIE Hideyuki, ABE Riichiro, AKIYAMA Masashi, SHIMIZU Hiroshi, Japanese journal of dermatology, 118, 5, 933, 937, 20 Apr. 2008
    日本皮膚科学会, Japanese
  • Development of an active disease model for bullous pemphigoid
    H. Ujiie, A. Shibaki, W. Nishie, G. Wang, Y. Tateishi, H. Nakamura, M. Akiyama, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 128, S10, S10, Apr. 2008
    English, Summary international conference
  • Novel therapeutic strategy for bullous pemphigoid using recombinant monoclonal fab antibody against NC16A domain of collagen XVII
    G. Wang, A. Shibaki, H. Ujiie, W. Nishie, Y. Tateishi, K. Kikuchi, Q. Li, J. R. McMilan, H. Morioka, D. Sawamura, H. Nakamura, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 128, S12, S12, Apr. 2008
    English, Summary international conference
  • 頭皮に生じた脂腺癌の1例
    冨田 幸希, 加藤 直子, 夏賀 健, 氏家 英之, 皮膚科の臨床, 50, 4, 539, 541, Apr. 2008
    金原出版(株), Japanese
  • 脂腺母斑に生じた皮膚腫瘍3症例についての検討               
    山根 尚子, 加藤 直子, 大澤 倫子, 柳 輝希, 氏家 英之, 日本皮膚科学会雑誌, 118, 1, 86, 86, Jan. 2008
    (公社)日本皮膚科学会, Japanese
  • Genomic ABCA12 mutations in ichthyosis patients with multiple skin malignant neoplasia
    K. Natsuga, M. Akiyama, N. Kato, K. Sakai, Y. Sugiyama-Nakagiri, M. Nishimura, H. Hata, M. Abe, K. Arita, Y. Tsuji-Abe, T. Onozuka, S. Aoyagi, K. Kodama, H. Ujiie, Y. Tomita, H. Shimizu, JOURNAL OF INVESTIGATIVE DERMATOLOGY, 127, S81, S81, Oct. 2007
    English, Summary international conference
  • 免疫グロブリン大量静注療法が著効した水疱性類天疱瘡の1例               
    新熊 悟, 阿部 由紀子, 冨田 幸希, 夏賀 健, 氏家 英之, 阿部 理一郎, 秋山 真志, 清水 宏, 皮膚の科学, 6, 3, 319, 319, Jun. 2007
    日本皮膚科学会-大阪地方会・京滋地方会, Japanese
  • タクロリムスが有効であったsclerodermatous chronic GVHDの1例
    冨田 幸希, 加藤 直子, 夏賀 健, 氏家 英之, 笠井 正晴, 臨床皮膚科, 61, 4, 318, 321, Apr. 2007
    (株)医学書院, Japanese
  • 水疱性類天疱瘡モデルマウス作製による病態解明               
    芝木 晃彦, 氏家 英之, 立石 八寿貴, 王 剛, 西江 渉, 澤村 大輔, 清水 宏, 日本皮膚科学会雑誌, 117, 4, 721, 721, Mar. 2007
    (公社)日本皮膚科学会, Japanese
  • 骨髄浸潤をきたしたMerkel細胞癌の1例               
    根本 育恵, 松村 和子, 藤田 靖幸, 近藤 真, 大西 勝憲, 夏賀 健, 氏家 英之, 冨田 幸希, 加藤 直子, 日本皮膚科学会雑誌, 117, 1, 69, 69, Jan. 2007
    (公社)日本皮膚科学会, Japanese
  • 巨大皮膚腫瘤より発見された後腹膜腔原発びまん性大細胞型B細胞リンパ腫の1例
    飯塚 さとし, 氏家 英之, 夏賀 健, 冨田 幸希, 加藤 直子, 鈴木 宏明, 山城 勝重, 臨床皮膚科, 60, 13, 1258, 1261, Dec. 2006
    (株)医学書院, Japanese
  • CD10が陽性を示したatypical fibroxanthomaの1例
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 皆川 英彦, 舟山 恵美, 國分 一郎, 臨床皮膚科, 60, 13, 1262, 1264, Dec. 2006
    (株)医学書院, Japanese
  • 組織球系マーカーが陽性であったCellular Neurothekeomaの1例
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 柳 輝希, 皮膚科の臨床, 48, 12, 1707, 1710, Nov. 2006
    金原出版(株), Japanese
  • 25年後に脳転移をきたした悪性黒色腫の1例
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 臨床皮膚科, 60, 11, 1052, 1054, Oct. 2006
    (株)医学書院, Japanese
  • 北海道がんセンターにおける過去12年間の乳房外Paget病16例の報告と本邦810例の文献的考察               
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 日本皮膚科学会雑誌, 116, 5, 815, 815, May 2006
    (公社)日本皮膚科学会, Japanese
  • 北海道がんセンターにおける過去12年間の皮膚付属器腫瘍の検討               
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 日本皮膚科学会雑誌, 116, 5, 822, 822, May 2006
    (公社)日本皮膚科学会, Japanese
  • 慢性痒疹が先行した悪性リンパ腫の1例               
    夏賀 健, 阿部 理一郎, 氏家 英之, 有田 賢, 芝木 晃彦, 澤村 大輔, 清水 宏, 日本皮膚科学会雑誌, 116, 4, 473, 473, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • M.fortuitumを検出した皮膚非定型抗酸菌症の1例               
    守内 玲寧, 氏家 英之, 有田 賢, 清水 忠道, 清水 宏, 南本 俊之, 日本皮膚科学会雑誌, 116, 4, 474, 474, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 悪性黒色腫の治療歴を有する非水疱型先天性魚鱗癬様紅皮症(NBCIE)患者に生じた末梢T細胞性リンパ腫(PTCL)               
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 日本皮膚科学会雑誌, 116, 4, 475, 475, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 膝窩リンパ節転移を生じたCK20陰性Merkel cell carcinomaの1例               
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 日本皮膚科学会雑誌, 116, 4, 475, 475, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • リンパ節転移をきたした外陰部apocrine ductal carcinomaの1例               
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 皆川 英彦, 舟山 恵美, 木村 中, 日本皮膚科学会雑誌, 116, 4, 478, 478, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 広範な皮膚浸潤を呈したPeripheral T-cell lymphoma,unspecified(PTCL-U)の1例               
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 深谷 徹, 相川 啓子, 日本皮膚科学会雑誌, 116, 4, 478, 478, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 頭皮に生じたsebaceous carcinomaの1例               
    冨田 幸希, 加藤 直子, 夏賀 健, 氏家 英之, 日本皮膚科学会雑誌, 116, 4, 479, 479, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 北海道がんセンターにおける過去12年間の乳房外Paget病16例の検討               
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 日本皮膚科学会雑誌, 116, 4, 483, 483, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 北海道がんセンターにおける過去12年間の皮膚付属器腫瘍の検討               
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 日本皮膚科学会雑誌, 116, 4, 483, 483, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 北海道がんセンターにおける過去12年間のBowen病およびBowen病由来進行癌44例の検討               
    夏賀 健, 加藤 直子, 氏家 英之, 冨田 幸希, 日本皮膚科学会雑誌, 116, 4, 486, 486, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 北海道がんセンターにおけるMerkel cell carcinoma 7例の検討               
    氏家 英之, 加藤 直子, 夏賀 健, 冨田 幸希, 皆川 英彦, 舟山 恵美, 日本皮膚科学会雑誌, 116, 4, 486, 486, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • エトレチナート全身投与を試みた皮膚硬化型慢性移植片対宿主病の1例               
    冨田 幸希, 加藤 直子, 夏賀 健, 氏家 英之, 笠井 正晴, 日本皮膚科学会雑誌, 116, 4, 489, 489, Apr. 2006
    (公社)日本皮膚科学会, Japanese
  • 著明な眼瞼腫脹および顔面神経麻痺を呈したsarcoidosis               
    八百坂 遵, 氏家 英之, 阿部 理一郎, 清水 宏, 日本皮膚科学会雑誌, 115, 6, 916, 916, May 2005
    (公社)日本皮膚科学会, Japanese
  • 臭化ブチルスコポラミン(ブスコパン)内服が奏効した難治性コリン性蕁麻疹               
    氏家 英之, 清水 忠道, 夏賀 健, 有田 賢, 冨澤 幸生, 北城 奈津子, 清水 宏, 日本皮膚科学会雑誌, 115, 3, 470, 470, Mar. 2005
    (公社)日本皮膚科学会, Japanese
  • 化膿性乳腺炎に合併した結節性紅斑の2例               
    氏家 英之, 澤村 大輔, 立石 八寿貴, 猪熊 大輔, 横田 浩一, 下川原 出, 清水 宏, 日本皮膚科学会雑誌, 114, 11, 1816, 1816, Oct. 2004
    (公社)日本皮膚科学会, Japanese
  • 免疫グロブリン大量静注療法による薬疹として生じた汗疱
    氏家 英之, 澤村 大輔, 立石 八寿貴, 芝木 晃彦, 清水 宏, 皮膚科の臨床, 46, 1, 89, 91, Jan. 2004
    金原出版(株), Japanese
  • シクロスポリン内服が奏効した大動脈炎症候群に合併した壊疽性膿皮症の1例               
    氏家 英之, 澤村 大輔, 横田 浩一, 西江 渉, 清水 宏, 日本皮膚科学会雑誌, 113, 10, 1582, 1582, Sep. 2003
    (公社)日本皮膚科学会, Japanese
  • 免疫グロブリン大量静注療法後に生じた汗疱の2例               
    氏家 英之, 澤村 大輔, 立石 八寿貴, 芝木 晃彦, 清水 宏, 日本皮膚科学会雑誌, 113, 10, 1577, 1578, Sep. 2003
    (公社)日本皮膚科学会, Japanese
  • 免疫グロブリン大量静注療法による汗疱               
    氏家 英之, 澤村 大輔, 立石 八寿貴, 芝木 晃彦, 清水 宏, 日本皮膚科学会雑誌, 113, 5, 804, 804, Apr. 2003
    (公社)日本皮膚科学会, Japanese
  • Raltitrexed(ZD 1694)の進行結腸・直腸癌に対する後期第II相臨床試験
    西庄 勇, 吉川 宣輝, 江端 俊彰, 長内 宏之, 氏家 重紀, 三田地 泰司, 筒井 光広, 沢井 清司, 西口 幸雄, 安武 晃一, 若杉 英之, 涌井 昭, 癌と化学療法, 27, 1, 81, 91, Jan. 2000
    (株)癌と化学療法社, Japanese
  • AIDSに関する学生の意識調査
    秋葉 均, 安藤 英之, 氏家 勇一, 福島医学雑誌, 44, 1~2, 43, 51, Jun. 1994
    福島医学会, Japanese
  • Banti症候群と誤診した巨大胃平滑筋腫の1症例               
    小川昭一郎, 氏家浄慧, 松野英之, 小林昇, 川島卓爾, 安藤忠夫, 平田利弘, 池田余彌, 金指恭三, 三島敏郎, 平野壽, 熊谷松作, 飛田愼一, 小坂三郎, 岩崎英雄, 日大医学雑誌, 21, 2, 326, 326, Feb. 1962
    日本大学医学会, Japanese

Research Themes

  • Development of a novel therapeutic strategy for angiosarcoma through drug screening
    Grants-in-Aid for Scientific Research
    01 Apr. 2023 - 31 Mar. 2026
    中村 秀樹, 柳 輝希, 氏家 英之
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 23K07757
  • 水疱性類天疱瘡におけるIgE自己抗体特異的皮膚炎症増幅機構の解明
    科学研究費助成事業
    01 Apr. 2022 - 31 Mar. 2025
    泉 健太郎, 氏家 英之
    1)水疱性類天疱瘡(B患者の末梢血中の活性化好塩基球上のBP-IgEに結合するBP180ペプチドを抗17型コラーゲン抗体で検出を図る。
    治療開始前の水疱性類天疱瘡患者の末梢血中好塩基球についてAllergenicity kitを用いてフローサイトメトリ―にて解析を行うと好塩基球の活性化マーカ-であるCD203cの発現が亢進していることを確認した。CD203cの発現が亢進している水疱性類天疱瘡患者の末梢血好塩基球に対して水疱性類天疱瘡自己抗体の主要な病原エピトープであるBP180のNC16A領域を標的とするマウスモノクローナル抗体(TS39-3)を蛍光標識した後、好塩基球表面のIgE自己抗体上に結合しているであろうBP180断片の検出を試みるもTS39-3特異的なシグナルを検出することはできなかった。IgE己抗体に補足されるBP180が全長や主要な病原エピトープであるNC16A領域ではない可能性が懸念された。


    2)寛解期にある水疱性類天疱瘡患者末梢血好塩基球をBP180リコンビナントタンパクで刺激し、好塩基球の活性化を指標にBP-IgEの検出・定量を試みる。
    治療を開始し、寛解導入後の水疱性類天疱瘡患者の末梢血をBP180全長リコンビナントタンパクを用いて刺激すると好塩基球のCD203c発現の亢進みられる症例を認めた。CD203cの発現亢進の程度を定量化するため、(BP180刺激-PBS)/(抗IgE刺激-PBS)をstimulation indexと定義した。stimulation indexがbullous pemphigoid disease area index (BPDAI)で表される客観的な皮疹の重症度に正の相関がみられる傾向を認めた。
    日本学術振興会, 基盤研究(C), 北海道大学, 22K08421
  • 加齢に着目した皮膚の免疫自己寛容破綻機序の解明
    科学研究費助成事業 基盤研究(B)
    01 Apr. 2021 - 31 Mar. 2024
    氏家 英之, 岩田 浩明, 泉 健太郎, 夏賀 健, 柳 輝希
    免疫系の老化、すなわち免疫老化によって自己免疫疾患のリスクが増加することが知られているが、詳細な機序の大部分は不明である。水疱性類天疱瘡(BP)は表皮基底膜部に存在するBP180やBP230に対する自己抗体によって生じる自己免疫性水疱症で、高齢者に好発する。我々は最近、一部の高齢マウスは表皮基底膜部に対する自己抗体を自然産生していることを発見した。本研究の目的は、BPをモデル疾患として“加齢に伴う免疫自己寛容の破綻機序を解明する”ことである。
    まず、高齢マウスが産生する抗基底膜部自己抗体の標的抗原を同定するため、BP180、ラミニンγ1、ラミニンγ2、ラミニンβ3、ラミニンα3の各リコンビナントタンパクを作成した。高齢マウス(70週齢以上)の血清を採取し、作成したリコンビナントタンパクを基質としてウエスタンブロットを施行した。BP180リコンビナントタンパクを基質としたウエスタンブロット法で、約半数の高齢マウス血清中に抗BP180 IgM抗体が検出された。ラミニンγ1、ラミニンγ2、ラミニンβ3、ラミニンα3を基質としたウエスタンブロット法は陰性だった。
    次に、加齢に伴いB細胞が抗基底膜部自己抗体を産生する機序を、制御性T細胞(Treg)とAge-associate B cells(ABCs)に着目して検討した。Treg欠損マウスでは野生型マウスに比較して、脾臓におけるABCsが増加していた。
    また、薬剤投与により高齢マウスに病原性自己抗体を誘導できるかどうかを、近年BPの発症誘因として注目されているDPP-4阻害薬と抗PD-1抗体に着目して検討している。現在、高齢マウスにこれらの薬剤を投与中だが、今のところBP自己抗体の有意な誘導は見られていない。
    日本学術振興会, 基盤研究(B), 北海道大学, 21H02938
  • エピトープスプレディング現象に着目した水疱性類天疱瘡の病態解明
    科学研究費助成事業 基盤研究(B)
    01 Apr. 2020 - 31 Mar. 2023
    西江 渉, 岩田 浩明, 泉 健太郎, 氏家 英之, 夏賀 健
    2020年度は、18名の水疱性類天疱瘡患者(DPP4阻害薬内服中の症例を含む)末梢血単核球中のB細胞をEBウイルスを用い形質転換した。樹立したLymphoblastoid cell lineの培養上清を用い、水疱性類天疱瘡抗原であるBP180タンパクへの自己抗体の反応性をELISA法で確認したところ、全ての患者さん由来細胞でIgGクラス自己抗体の反応性は確認できなかった。しかし、6名のLymphoblastoid cell line培養上清に坑BP180 IgMクラス自己抗体を確認したため、形質転換した細胞群から単一あるいはポリクローナルな状態での、坑BP180自己抗体産生細胞の採取をFACSソーティングで試みた。その結果、坑BP180自己抗体産生細胞数が極めて少なかったことと、蛍光標識BP180タンパクが細胞表面に結合した後のエンドサイトーシスに伴う蛍光の減弱のため、細胞の単離は上手くいかなかった。そこで、pH耐性緑色蛍光タンパクであるGamillusを融合したBP180タンパクを作製し、再度FACSソーティングを行うこととした。細胞を単離したのち、次世代シークエンサーによる自己抗体可変領域のアミノ酸配列の解読と、リコンビナント坑BP180自己抗体の作製、そしてマウスへ投与し病原性の確認を計画しそれぞれの予備実験を開始していたが、今後の研究を遂行できなくなったため、2021年以降の交付申請は辞退することにした。
    日本学術振興会, 基盤研究(B), 北海道大学, 20H03700
  • Elucidation of the mechanism of breakdown of cutaneous immune tolerance using an innovative mouse model
    Grants-in-Aid for Scientific Research
    30 Jul. 2020 - 31 Mar. 2022
    Ujiie Hideyuki
    To elucidate the mechanism by which BP180, a bullous pemphigoid antigen, is a likely target of autoimmunity, we focused our analysis on central immune tolerance. Since the mRNA expression of BP180 in thymic medullary epithelial cells (mTECs) was not lower than that of other proteins, a loss of central immune tolerance of T cells was considered less likely to be involved as a mechanism of BP180 immune tolerance failure. In addition, Treg-deficient/K14-BP180-humanized mice, in which human BP180 was forced to be expressed in the basement membrane and mTECs by the K14 promoter and the Foxp3 gene was deleted, did not produce anti-human BP180 antibodies, suggesting that forced self-antigen expression in mTECs may rescue the breakdown of peripheral immune tolerance. The results suggest that forced expression of autoantigen in mTECs may be able to rescue the disruption of peripheral immune tolerance.
    Japan Society for the Promotion of Science, Grant-in-Aid for Challenging Research (Exploratory), Hokkaido University, 20K21590
  • The impact of complement in blistering formation of pemphigoid
    Grants-in-Aid for Scientific Research
    01 Apr. 2019 - 31 Mar. 2022
    Iwata Hiroaki
    The inflammation caused by complement is thought to be essential for the blistering mechanism of bullous pemphigoid. However, we have previously shown the complement independent blstering mechanism. At this study, we found that C1q which is a component of comlement systems enhaces the endocytosis of BP180 after binding BP-IgG to BP180 using cell culture system. In additon, keratinocytes stimulated with BP-IgG produced C1q from from keratinocytes and enhaced the endocytosis of BP180. Based on these results, we think that the complement may directly modulate the pathogenicity of BP-IgG autoantibody. In addition, complement ennhance the severity of buloous pemphigid though the inflammation.
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 19K08786
  • Generation of bullous pemphigoid antigen specific T cell clones
    Grants-in-Aid for Scientific Research Grant-in-Aid for Scientific Research (B)
    01 Apr. 2017 - 31 Mar. 2020
    UJIIE Hideyuki
    Bullous pemphigoid (BP) is a most common autoimmune blistering disorder, which preferentially affects elderly people. It is caused by autoantibodies to type XVII collagen (COL17, BP180) at the dermal-epidermal junction of the skin. Although autoreactive T cells is important to produce autoantibody, the pathogenicity of COL17-reactive T cells is still largely unclear. In this study, we have successfully established human COL17-reactive T cell lines by stimulating and culturing lymphocytes obtained from human COL17-immunized mice. Those T cell lines are CD4+ effector T cells which produce IFN-gamma and showed weak pathogenicity in a mouse model.
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (B), Hokkaido University, 17H04238
  • Novel bullous pemphigoid models induced by breaking tolerance to autoantigens.
    Grants-in-Aid for Scientific Research Grant-in-Aid for Scientific Research (B)
    01 Apr. 2016 - 31 Mar. 2019
    Nishie Wataru, IZUMI kentaro, TOYONAGA ellen
    The aim of this study is to develop bullous pemphigoid models for elucidating the pathomechanisms and producing novel therapies. We have established several novel bullous pemphigoid models by immunizing recombinant collagen XVII (COL17) proteins. Notably, monoclonal antibodies derived from the immunized mice disclosed that epitopes and subclasses of the monoclonal antibodies were associated with phenotypes of skin disease.
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (B), Hokkaido University, 16H05365
  • The role of Th17 cells in the pathogenesis of autoimmune blistering disease
    Grants-in-Aid for Scientific Research Grant-in-Aid for Scientific Research (C)
    01 Apr. 2016 - 31 Mar. 2019
    Iwata Hiroaki
    We tried the establishment of the autoimmune blistering disease mouse model induced by epicutaneous immunization instead of subctaneous injection. In this process, imiquimod cream was applied onto the mouse back skin, and then antigen protein was applied on the inflamed skin. Imiquimod cream is known to activate dendritic cell in the skin and induce Th17 cells. Compared to conventional subctaneous immunization with adjuvant, epicutaneous immunization induced the autoaintibodies less frequently and also less titier levels. However, epicutaneous immunization without adjuvant could induce autoantibodies in several mice.
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 16K10141
  • Analysis of regulatory T cells and application for cell therapy in bullous pemphigoid
    Grants-in-Aid for Scientific Research Grant-in-Aid for Young Scientists (B)
    01 Apr. 2015 - 31 Mar. 2017
    Ujiie Hideyuki, MURAMATSU KEN
    Regulatory T cells (Treg) are a T cell subset which play an important role on the self-tolerance. The master transcription factor of Treg is Foxp3, and its gene mutations induce severe systemic autoimmunity in mice (scurfy mice) and humans (IPEX syndrome). This study demonstrates that the absence of Tregs induce autoantibodies to epidermal basement membrane zone. We further revealed that the autoantibodies in scurfy mice and IPEX patients target bullous pemphigoid antigens, BP230 and type XVII collagen. These results suggest that a dysfunction of Tregs may trigger the development of bullous pemphigoid.
    Japan Society for the Promotion of Science, Grant-in-Aid for Young Scientists (B), Hokkaido University, 15K19667
  • Production of a novel blistering disease model by regulating expression of collagen XVII
    Grants-in-Aid for Scientific Research Grant-in-Aid for Scientific Research (B)
    01 Apr. 2012 - 31 Mar. 2016
    Nishie Wataru, UJIIE HIDEYUKI, SHINKUMA SATORU, NATSUGA KEN
    Collagen XVII (COL17) is a hemidesmosomal molecule which keeps adhesion between the epidermis and dermis. Loss of CO17 expression due to mutation in the COL17A1 leads to blistering disease, epidermolysis bullosa (EB); in addition, autoimmunity to COL17 results in autoimmune blistering disorder bullous pemphigoid (BP). The purpose of this study is to produce a novel blistering model by regulating COL17 expression based on Tet-on system. Transgenic mice carrying human COL17 cDNA driven under the CMV promoter with Tet response element and Tet transactivator sequences driven under the K14 promoter were produced. Upon oral administration of doxycycline (DOX), the Tg mice started to express human COL17 in basal keratinocytes, following by production of IgG autoantibodies directing human COL17. The Tg mice must be useful tool for elucidating pathogenesis of EB and BP, as well as for establishing therapies for these orphan diseases.
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (B), Hokkaido University, 24390274
  • Development of novel treatment targeting complement for bullous pemphigoid
    Grants-in-Aid for Scientific Research Grant-in-Aid for Scientific Research (C)
    2011 - 2013
    SHIBAKI Akihiko, SHIMIZU Hiroshi, UJIIE Hideyuki
    Complement activation and subsequent recruitment of inflammatory cells at the dermal-epidermal junction are believed to be essential for blister formation in bullous pemphigoid (BP), an autoimmune blistering disease induced by autoantibodies against COL17; however, the involvement of complement-independent pathways has recently been proposed. To directly examine the necessity of the complement activation in blister formation, we generated C3-deficient COL17-humanized mice and monoclonal antibodies against COL17 with different ability of complement activation. By using them, we demonstrate that the deposition of antibodies, and not complements, is relevant to the induction of blister formation. BP autoantibodies reduced the amount of COL17 in cultured normal human keratinocytes, and the COL17-depletion was associated with a ubiquitin-proteasome pathway. In conclusion, the COL17-depletion induced by BP autoantibodies, and not complement activation, is essential for the blister formation.
    Japan Society for the Promotion of Science, Grant-in-Aid for Scientific Research (C), Hokkaido University, 23591635
  • Analysis of the role of T cell subsets and the development of novel treatment for bullous pemphigoid
    Grants-in-Aid for Scientific Research Grant-in-Aid for Young Scientists (B)
    2011 - 2012
    UJIIE Hideyuki
    We analyzed the role of T cell subsets in bullous pemphigoid (BP) by using active BP mouse model and cytokine knockout (KO) mice. We generated active BP mouse model by using IL-12KO mice and IL-4KO mice. BP model mice that received IL-12KO splenocytes showed strong deposition of IgG1 (Th2-type reaction) and BP model mice that received IL-4KO splenocytes showed strong deposition of IgG2 (Th1-type reaction), while the deposition of complement and skin disease were almost same in both models. These results indicate that cytokine KO induce the difference in IgG subclass but fail to induce the difference in the amount of IgG-production, disease severity or complement activation in BP model.
    Japan Society for the Promotion of Science, Grant-in-Aid for Young Scientists (B), Hokkaido University, 23791242