Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)

Hideyuki Ujiie, Hiroaki Iwata, Jun Yamagami, Takekuni Nakama, Yumi Aoyama, Shigaku Ikeda, Norito Ishii, Keiji Iwatsuki, Michiko Kurosawa, Daisuke Sawamura, Akiko Tanikawa, Daisuke Tsuruta, Wataru Nishie, Wataru Fujimoto, Masayuki Amagai, Hiroshi Shimizu

Research output: Contribution to journalArticlepeer-review

53 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)1102-1135
Number of pages34
JournalJournal of Dermatology
Issue number12
Publication statusPublished - 2019 Dec


  • autoimmune blistering diseases
  • bullous pemphigoid
  • epidermolysis bullosa acquisita
  • mucous membrane pemphigoid
  • treatment algorithm

ASJC Scopus subject areas

  • Dermatology


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