获得性大疱性表皮松解症合并症的研究进展  

Complications of epidermolysis bullosa acquisita

作　　者：李思哲[1] 左亚刚[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院皮肤科,北京100730

出　　处：《国际皮肤性病学杂志》2017年第4期205-207,共3页International Journal of Dermatology and Venereology

基　　金：北京市自然科学基金（7132203）

摘　　要：获得性大疱性表皮松解症是一种自身免疫性大疱性皮肤病，其发病与真皮一表皮连接的锚丝主要成分Ⅶ型胶原的自身免疫有关。获得性大疱性表皮松解症病变可累及皮肤和黏膜，主要表现多样的表皮下水疱，愈后可留有粟丘疹和萎缩性瘢痕。近年来，越来越多的研究证实，该病可合并其他皮肤病以及其他系统性疾病，包括消化系统疾病、自身免疫性疾病、内分泌系统疾病、感染性疾病、血液系统疾病以及肿瘤性疾病。部分合并疾病仅为个例报道，而另一些则有相关机制的研究。Epidermolysis bullosa acquisita （EBA） is a rare kind of autoimmune blistering disease associated with autoimmunity to type Ⅶcollagen, which is the major component of anchoring fibrils at the dermal-epidermal junction. EBA may involve the mucous membrane and skin, and mainly manifests as diverse subepidermal blisters leaving milia and atrophic scars after healing. Recently, it has been described in association with a number of other skin and systemic diseases, including gastrointestinal diseases, autoimmune diseases, endocrine diseases, infectious diseases, hematological diseases and neoplastic diseases. Some of the complications were described in case reports, and some were investigated about the relevant pathogenesis.

关 键 词：获得性大疱性表皮松解 免疫系统疾病 血液病 癌 皮肤疾病 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]