Amagai Masayuki, Tanikawa Akiko, Shimizu Tomoko, Hashimoto Takashi, Ikeda Shigaku, Kurosawa Michiko, Niizeki Hironori, Aoyama Yumi, Iwatsuki Keiji, Kitajima Yasuo
J Dermatol. 2014 Jun;41(6):471-86. doi: 10.1111/1346-8138.12486.
The Committee for Guidelines for the Management of Pemphigus was organized as one element of the Japanese Dermatological Association (JDA) and the Ministry of Health, Labour, and Welfare (MHLW) Research Project on Measures for Research Committee for Intractable Skin Disease. Pemphigus has been defined as a group of intractable autoimmune blistering diseases caused by anti-desmoglein 1 and/or anti-desmoglein 3 IgG autoantibodies by the MHLW. The diagnosis of this condition and the criteria for assessing its severity are based on suggestions from the MHLW Research Group. The clinical practice guidelines presented here are those that are currently recommended in Japan. However, symptoms and complications can vary widely among individual pemphigus patients, so not all therapies will be required to be in complete agreement with these guidelines.
天疱疮管理指南委员会是作为日本皮肤病学会（JDA）和厚生劳动省（MHLW）难治性皮肤病研究对策研究项目的一部分而组建的。厚生劳动省将天疱疮定义为由抗桥粒芯糖蛋白1和/或抗桥粒芯糖蛋白3 IgG自身抗体引起的一组难治性自身免疫性水疱病。该病的诊断及严重程度评估标准基于厚生劳动省研究小组的建议。此处呈现的临床实践指南是目前日本所推荐的。然而，天疱疮患者个体之间的症状和并发症差异很大，因此并非所有治疗都必须完全符合这些指南。
