营养不良型大疱表皮松解症的治疗研究进展  

Research progress in the treatment of dystrophic epidermolysis bullosa

作　　者：庄滨瑜 栗玉珍[1] 

机构地区：[1]哈尔滨医科大学附属第二医院皮肤科,哈尔滨150086

出　　处：《实用皮肤病学杂志》2016年第2期127-129,132,共4页Journal of Practical Dermatology

摘　　要：营养不良型大疱表皮松解症(dystrophic epidermolysis bullosa,DEB)是一种罕见的遗传性疾病,它是一类由于Ⅶ型胶原基因突变或缺失所引起的机械性大疱性疾病,其特征是皮肤水疱及瘢痕的形成,慢性、难以愈合的创伤。根据遗传方式可分为显性遗传营养不良型大疱表皮松解症(dominant dystrophic epidermolysis bullosa,DDEB)和隐性遗传营养不良型大疱表皮松解症(recessive dystrophic epidermolysis bullosa,RDEB)。近年来随着DEB遗传基因、分子发病机制及VII型胶原的结构、功能等方面的研究,DEB的治疗有了很多新的研究成果,有的已经进行临床前试验及临床试验。该文对DEB目前的治疗方法及其新进展进行概述。Dystrophic epidermolysis bullosa is a rare genetic disease, which is also a kind of mechanical bullous disease caused by the human body type VII collagen gene mutation or loss, characterized by skin blisters and scar formation, chronic and diffi cult to heal wounds. According to the genetic type, the disease can be divided into dominant dystrophic epidermolysis bullosa and recessive dystrophic epidermolysis bullosa. In recent years, with the development of the study of DEB genetic and molecular pathogenesis, structure and function of type VII collagen, DEB treatment has gained a lot of new research results, some of which have carried out preclinical tests and clinical trials. In this paper, present treatment methods and new progress of DEB are summarized.

关 键 词：大疱表皮松解症 营养不良 Ⅶ型胶原 治疗 

分 类 号：R758.59[医药卫生—皮肤病学与性病学]