家族性慢性良性天疱疮的诊治进展  

Diagnosis and Treatment of Familial Chronic Benign Pemphigus

作　　者：夏颖 陈思远 XIA Ying;CHEN Siyuan(Department of Dermatology,Tongji Medical College Huangzhong University of Science and Technology,Wuhan 430030,China;Department of Dermatology,Union Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology,Wuhan 430022,China)

机构地区：[1]华中科技大学同济医学院附属同济医院皮肤科,湖北武汉430030 [2]华中科技大学同济医学院附属协和医院皮肤科,湖北武汉430022

出　　处：《皮肤科学通报》2020年第1期100-105,共6页Dermatology Bulletin

摘　　要：家族性慢性良性天疱疮(familial chronic benign pemphigus,FCBP),又称Hailey-Hailey病,是一种少见的常染色体显性遗传性疾病。其临床特征表现为边界清楚的红斑、结痂,伴烧灼感或瘙痒感。皱褶及易摩擦处是最常见的受累部位,颈部也经常累及。家族性慢性良性天疱疮临床上治疗困难、易误诊,本文就近年来该病的发病机制、临床表现及组织病理特点、诊断及鉴别诊断、治疗等方面做一综述。Familial chronic benign pemphigus(FCBP),also known as Hailey-Hailey disease(HHD),is an autosomal dominant dermatosis characterized by acantholysis of the skin.The disease is characterized by repeated blisters and bullae,which are prone to burst and form erosion.Most commonly involved sites are the neck,axillae,and groins.We reviewed the pathogeny,clinical manifestations,differential diagnosis and management of familial chronic benign pemphigus.

关 键 词：家族性慢性良性天疱疮 发病机制 临床表现 治疗 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]