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한국인의 항p200 유사천포창 환자의 임상조직학적 특징에 대한 후향적 단일기관 연구

Other Titles
 Clinicopathological Characteristics of Patients with Anti-p200 Pemphigoid in Korea: A Retrospective Single-Center Study 
 김태희  ;  Norito Ishii  ;  Takashi Hashimoto  ;  김수찬  ;  김종훈 
 Korean Journal of Dermatology(대한피부과학회지), Vol.60(8) : 476-482, 2022-09 
Journal Title
Korean Journal of Dermatology(대한피부과학회지)
Issue Date
Anti-laminin γ1 pemphigoid ; Anti-p200 pemphigoid
Background: Anti-p200 pemphigoid (anti-laminin γ1 pemphigoid) is a rare subepidermal autoimmune blistering disease. Autoantibodies against a 200 kDa protein should be identified by immunoblot assay using dermal extracts for the diagnosis of anti-p200 pemphigoid. So far, approximately 100 cases have been reported worldwide; however, in Korea, only a few case reports have been described in the literature.
Objective: This study aimed to analyze the clinicopathological findings of patients with anti-p200 pemphigoid in Korea.
Methods: This was a single-center retrospective study. Medical records of patients with anti-p200 pemphigoid who were diagnosed between 2004 and 2021 were reviewed.
Results: Twelve patients were included in this study. There were eight male and four female. Mean age of disease onset was 61.4 years. Only one patient had a history of psoriasis. Although the distribution of lesions varied, most patients presented with multiple erythematous rashes and blisters on their hands and feet. There were four cases of mucosal involvement. Histopathological examination revealed subepidermal blistering with neutrophilic and/or eosinophilic infiltration. All the patients were treated with systemic corticosteroids. The adjuvant therapies applied were colchicine (66.7%), dapsone (58.3%), doxycycline (41.7%), mycophenolate mofetil (16.7%), intravenous immunoglobulin, and rituximab (8.3%). The remission rate was 75% with an average time of 10 months to reach remission, and two patients experienced relapse during treatment.
Conclusion: This is the first retrospective study to analyze anti-p200 pemphigoid therapy in Korea. Clinically, vesicles on the extremities are characteristic of anti-p200 pemphigoid. Most patients showed a good response to the conventional treatment.
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1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Kim, Jong Hoon(김종훈) ORCID logo https://orcid.org/0000-0002-3385-8180
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