Junctional epidermolysis bullosa in children:an overview  

作　　者：Smitha Parameswaran Namboothiri 

机构地区：[1]Gastro Investigations Suite,Great Ormond Street Hospital for Children NHS Foundation Trust,London WC1N 3JH,United Kingdom.

出　　处：《Clinical Research Communications》2023年第1期5-9,共5页临床研究通讯

摘　　要：Epidermolysis bullosa consist of a pattern of diseases which is mainly associated with genetic defects in the integrity of structures that cause the adhesion of the epidermis with the dermis,primarily called as the Basement Membrane Zone.If the defect is associated with the lamina lucida of the basement membrane zone,it is called junctional epidermolysis bullosa(JEB).JEB is mainly inherited in an autosomal recessive manner.The characteristic feature of all the JEB subtypes is enamel hypoplasia.This article is aimed at identifying the main features of JEB in children.Fifty articles which were published between 2000 and 2022 were reviewed and the types,investigations and management of JEB are explained based on the existing literature.

关 键 词：junctional epidermolysis bullosa lamina lucida enamel hypoplasia 

分 类 号：TG1[金属学及工艺—金属学]