한국인의 항p200 유사천포창 환자의 임상조직학적 특징에 대한 후향적 단일기관 연구 (Clinicopathological Characteristics of Patients with Anti-p200 Pemphigoid in Korea: A Retrospective Single-Center Study)

서지정보

· 발행기관 : 대한피부과학회
· 수록지 정보 : 대한피부과학회지 / 60권 / 8호 / 476 ~ 482페이지
· 저자명 : 김태희, Norito Ishii, Takashi Hashimoto, 김수찬, 김종훈

초록

Background: Anti-p200 pemphigoid (anti-laminin γ1 pemphigoid) is a rare subepidermal autoimmune blisteringdisease. Autoantibodies against a 200 kDa protein should be identified by immunoblot assay using dermal extractsfor 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 inKorea.
Methods: This was a single-center retrospective study. Medical records of patients with anti-p200 pemphigoid whowere 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 diseaseonset was 61.4 years. Only one patient had a history of psoriasis. Although the distribution of lesions varied, mostpatients presented with multiple erythematous rashes and blisters on their hands and feet. There were four cases ofmucosal involvement. Histopathological examination revealed subepidermal blistering with neutrophilic and/oreosinophilic infiltration. All the patients were treated with systemic corticosteroids. The adjuvant therapies appliedwere colchicine (66.7%), dapsone (58.3%), doxycycline (41.7%), mycophenolate mofetil (16.7%), intravenousimmunoglobulin, and rituximab (8.3%). The remission rate was 75% with an average time of 10 months to reachremission, 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 theconventional treatment.

영어초록

Background: Anti-p200 pemphigoid (anti-laminin γ1 pemphigoid) is a rare subepidermal autoimmune blisteringdisease. Autoantibodies against a 200 kDa protein should be identified by immunoblot assay using dermal extractsfor 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 inKorea.
Methods: This was a single-center retrospective study. Medical records of patients with anti-p200 pemphigoid whowere 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 diseaseonset was 61.4 years. Only one patient had a history of psoriasis. Although the distribution of lesions varied, mostpatients presented with multiple erythematous rashes and blisters on their hands and feet. There were four cases ofmucosal involvement. Histopathological examination revealed subepidermal blistering with neutrophilic and/oreosinophilic infiltration. All the patients were treated with systemic corticosteroids. The adjuvant therapies appliedwere colchicine (66.7%), dapsone (58.3%), doxycycline (41.7%), mycophenolate mofetil (16.7%), intravenousimmunoglobulin, and rituximab (8.3%). The remission rate was 75% with an average time of 10 months to reachremission, 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 theconventional treatment.

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