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한국인의 항p200 유사천포창 환자의 임상조직학적 특징에 대한 후향적 단일기관 연구
김태희,Norito Ishii,Takashi Hashimoto,김수찬,김종훈 대한피부과학회 2022 대한피부과학회지 Vol.60 No.8
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.
Clinical outcome and lymphocyte kinetics in IgA pemphigus treated with rituximab
( Seung-ju Lee ),( Ayeong Lee ),( Norito Ishii ),( Takashi Hashimoto ),( Gwang Seong Choi ),( Soo-chan Kim ),( Jong Hoon Kim ) 대한피부과학회 2021 대한피부과학회 학술발표대회집 Vol.72 No.2
IgA pemphigus is an IgA-mediated autoimmune blistering disease characterized by IgA autoantibody deposition in the intercellular space of the epidermis. Herein, we demonstrated rituximab efficacy in IgA pemphigus by showing decreases of IgA+ antibody-secreting cells. A 21-year-old woman presented with a 2-year history of extensive pruritic vesiculopustular eruption over the trunk and thighs. Histopathologic findings showed intraepidermal separation containing neutrophils and eosinophils. Direct and indirect immunofluorescence tests showed IgA deposition in the intercellular space of the epidermis. Based on clinical, histologic, and immunopathologic findings, the patient was diagnosed with IgA pemphigus. She continuously experienced relapse on systemic corticosteroid, colchicine, dapsone, and mycophenolate mofetil over the 2 years. We started two infusions of 1000 mg rituximab separated by 2 weeks to control her disease. Three months after rituximab treatment, she stopped using systemic corticosteroids and achieved complete remission. She has remained in complete remission for 10 months. Correlating with her clinical remission, the IgA+ B cell frequency was depleted after rituximab treatment, and the IgA+ plasma cell frequency in the CD20- cells markedly decreased, although most plasma cells were IgA+ after rituximab. Here, we present a feasible role of rituximab in a recalcitrant IgA pemphigus case refractory to conventional treatment.