한국인의 항p200 유사천포창 환자의 임상조직학적 특징에 대한 후향적 단일기관 연구

Abstract

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.