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

被引：66

作者：

Ujiie, Hideyuki ^{[1
]}

Iwata, Hiroaki ^{[1
]}

Yamagami, Jun ^{[2
]}

Nakama, Takekuni ^{[3
]}

Aoyama, Yumi ^{[4
]}

Ikeda, Shigaku ^{[5
]}

Ishii, Norito ^{[3
]}

Iwatsuki, Keiji ^{[6
]}

Kurosawa, Michiko ^{[7
]}

Sawamura, Daisuke ^{[8
]}

Tanikawa, Akiko ^{[2
]}

Tsuruta, Daisuke ^{[9
]}

Nishie, Wataru ^{[1
]}

Fujimoto, Wataru ^{[4
]}

Amagai, Masayuki ^{[2
]}

Shimizu, Hiroshi ^{[1
]}

机构：

[1] Hokkaido Univ, Grad Sch Med, Dept Dermatol, Sapporo, Hokkaido, Japan

[2] Keio Univ, Sch Med, Dept Dermatol, Tokyo, Japan

[3] Kurume Univ, Sch Med, Dept Dermatol, Kurume, Fukuoka, Japan

[4] Kawasaki Med Sch, Dept Dermatol, Kurashiki, Okayama, Japan

[5] Juntendo Univ, Sch Med, Dept Dermatol, Tokyo, Japan

[6] Okayama Univ, Dept Dermatol, Grad Sch Med Dent & Pharmaceut Sci, Okayama, Japan

[7] Juntendo Univ, Fac Med, Dept Epidemiol & Environm Hlth, Tokyo, Japan

[8] Hirosaki Univ, Grad Sch Med, Dept Dermatol, Hirosaki, Aomori, Japan

[9] Osaka City Univ, Grad Sch Med, Dept Dermatol, Osaka, Japan

来源：

JOURNAL OF DERMATOLOGY | 2019年 / 46卷 / 12期

关键词：

autoimmune blistering diseases; bullous pemphigoid; epidermolysis bullosa acquisita; mucous membrane pemphigoid; treatment algorithm; LINKED-IMMUNOSORBENT-ASSAY; INTRAVENOUS IMMUNOGLOBULIN THERAPY; GESTATIONIS AUTOANTIBODIES RECOGNIZE; MYCOPHENOLATE-MOFETIL; VII COLLAGEN; CLINICAL-RESPONSE; PLASMA-EXCHANGE; IMMUNOSUPPRESSIVE THERAPY; TOPICAL CORTICOSTEROIDS; RETROSPECTIVE ANALYSIS;

D O I：

10.1111/1346-8138.15111

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

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.

引用

页码：1102 / 1135

页数：34

共 196 条

[1] Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non-responsive to conventional therapy: clinical outcome and post-treatment long-term follow-up [J].