获得性大疱性表皮松解症  

Epidermolysis Bullosa Acquisita

作　　者：姚南 廖洁月[1] 郭子瑜 张桂英[1] YAO Nan;LIAO Jieyue;GUO Ziyu;ZHANG Guiying(The Second Xiangya Hospital,Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院,湖南长沙410011

出　　处：《皮肤科学通报》2021年第3期228-232,共5页Dermatology Bulletin

摘　　要：获得性大疱性表皮松解症(Epidermolysis Bullosa Acquisita,EBA)是一种罕见的获得性的自身免疫性表皮下大疱病,血中存在抗VⅡ型胶原(COL7)的自身抗体,其临床表现多样,类似于遗传性营养不良性大疱性表皮松解症(Dystrophic Epidermolysis Bullosa,IDEB)或类天疱疮(Bullous Pemphigoid,BP),并可累及粘膜、指甲以及毛发,而且病程慢性,迁延不愈,目前诊断及治疗均较棘手。Epidermolysis Bullosa Acquisita(EBA)is a rare,acquired,auto-immune subepidermal blistering disease characterized by autoantibodies against type VⅡ collagen(COL7)in the blood.EBA has various clinical manifestations,similar to Inherited Dystrophic Epidermolysis Bullosa(IDEB)or Bullous Pemphigoid(BP).Besides the skin,mucosal epithelia,nail and hair can also be affected.The course of EBA is usually chronic and persistent.So far,the diagnosis and treatment of EBA are difficult.

关 键 词：获得性大疱性表皮松解症 抗VⅡ型胶原(COL7) 临床表现 诊断标准 治疗 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]