大疱性鱼鳞病及维A酸相关作用研究进展  

Advances on Epidermolytic Hyperkeratosis and Therapeutic Mechanism with Retinoids

作　　者：何玉清[1] 张锡宝[1] 曾抗[2] 

机构地区：[1]广州市皮肤病防治所,510095 [2]南方医科大学南方医院

出　　处：《国际皮肤性病学杂志》2006年第6期377-379,共3页International Journal of Dermatology and Venereology

基　　金：广东省自然科学基金资助(04004285)

摘　　要：大疱性鱼鳞病是一种少见的常染色体显性遗传性皮肤病,临床以皮肤过度角化为主要表现,并有3种临床亚型。其发病主要由K1、K10和K2e角蛋白基因突变引起,为常染色体显性遗传。临床试验证实,多数患者在局部或系统使用维A酸后,皮肤状态明显改善。就大疱性鱼鳞病的病因、发病机制和维A酸相关作用机制进行综述。Epidermolytic hyperkeratosis is a rare autosome dominantly inherited skin disorder with the presentations of erythroderma and hyperkeratosis. Mutations have been found in the genes of keratin 1 （K1）, K10 or K2c. The clinical studies confirm that the skin lesions obviously improve with systemic or topical retinoids. The etiology, pathogenesis, and therapeutic mechanism of retiniods in epidermolytic hyperkeratosis are reviewed.

关 键 词：角化过度症 表皮松解 维A酸类 角蛋白 

分 类 号：R758.52[医药卫生—皮肤病学与性病学]