Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)
Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)
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DOI：
10.1111/1346-8138.15111
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发表时间：
2019-10-24
影响因子：
3.1
通讯作者：
Shimizu, Hiroshi
中科院分区：
文献类型：
--
作者：
Ujiie, Hideyuki;Iwata, Hiroaki;Shimizu, Hiroshi
类天疱疮是一类自身免疫性表皮下水疱性疾病，其中自身抗体存款线性地沉积在表皮基底膜区（BMZ）。由免疫球蛋白G自身抗体介导的类天疱疮的主要亚型有大疱性类天疱疮（BP）、粘膜类天疱疮（MMP）和获得性大疱性表皮病（EBA）。为了制定日本皮肤病学会批准的首个类天疱疮疾病管理指南，作为厚生劳动省难治性疾病研究项目下罕见难治性皮肤病研究小组的一部分，成立了类天疱疮疾病（包括EBA）管理指南委员会。这些指南旨在为日本的BP、MMP和EBA管理提供最新信息。指南以证据为基础，总结了临床和免疫学表现、病理生理学、诊断标准、疾病严重程度判定标准、治疗算法和治疗建议。由于这些疾病的罕见性，很少有具有高度证据的临床研究，因此这些指南的几个部分是根据委员会的意见制定的。为了进一步优化这些指南，有必要根据新的证据进行定期修订。
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.