黏膜类天疱疮的研究进展  

Update on Mucous Membrane Pemphigoid

作　　者：王媛 李锁 冯素英 WANG Yuan;LI Suo;FENG Suying(Institute of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China)

机构地区：[1]中国医学科学院皮肤病医院(皮肤病研究所),北京协和医学院,江苏南京210042

出　　处：《中国皮肤性病学杂志》2021年第9期1050-1053,共4页The Chinese Journal of Dermatovenereology

基　　金：中国医学科学院医学与健康科技创新工程(CIFMS-2017-I2M-1-017);南京市国家级临床医学中心培育计划(2019060001)。

摘　　要：黏膜类天疱疮是指一组慢性、异质性、自身免疫性表皮下水疱病,主要累及口腔、眼睛等黏膜,其特点是具有形成瘢痕的风险。患者体内存在抗基底膜带的自身抗体,分别是针对BP180、BP230、层粘连蛋白332、整合素α6β4,和Ⅶ型胶原,其中BP180的羧基端、层粘连蛋白332被认为是主要的自身抗原。诊断主要基于临床表现、组织病理及免疫学检查。治疗上应根据受累部位,疾病严重程度及进展速度,选择合适的治疗方案,为了尽量减少不可逆的损害,多学科合作诊疗是必要的。Mucous membrane pemphigoid(MMP)is a heterogeneous group of chronic autoimmune subepithelial blistering diseases,predominantly affecting oral and ocular mucosae and is characterized by a risk for scarring.MMP is caused by autoantibodies against various autoantigens in the basement membrane zone,including BP180,BP230,laminin 332,integrinα6β4,and collagenⅦ.Of these,the C-terminus of BP180 and laminin 332 are thought to be major autoantigens for MMP.The diagnosis of MMP is based on the clinical presentation,histological and immunopathological findings.The appropriate approach to therapy is influenced by the following clinical factors:site(s)of involvement,severity of disease,rapidity of progression.Multidisciplinary management is often needed to minimize risk for adverse sequelae of this disease.

关 键 词：黏膜类天疱疮 BP180 层粘连蛋白332 整合素Α6Β4 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]