遗传性大疱性表皮松解症口腔粘膜损害特点分析  

Oral manifestations of the epidermolysis bullosa

作　　者：杨建强[1] 杨卫平[2] 

机构地区：[1]湖州师范学院医学院门诊部皮肤科,浙江湖州313000 [2]湖州师范学院附属第一医院口腔科,浙江湖州313000

出　　处：《中国优生与遗传杂志》2012年第2期129-130,125,共3页Chinese Journal of Birth Health & Heredity

摘　　要：目的了解遗传性大疱性表皮松解症(EB)的口腔粘膜损害临床特点。方法检索近20年来文献发表的具有详细口腔粘膜损害描述的36例患者,分析患者的口腔粘膜损害及其相关临床特点。结果 (1)EB患者可出现散在的口腔糜烂;或频发口腔糜烂和溃疡;或深在性口腔溃疡伴严重瘢痕和挛缩,重者可见伸舌受限和咀嚼困难。(2)具有口腔粘膜损害的EB患者中,以营养不良型(DEB)居多,占所有患者的66.7%;其中又以常染色体隐性遗传营养不良型(RDEB)居多;(3)不同亚型患者的口腔粘膜损害程度可不等,RDEB患者具有较严重的口腔粘膜损害。不同家系中患者、同一家系中患者之间的口腔粘膜损害程度存在差异性。结论遗传性大疱性表皮松解症除皮肤、指趾甲损害外,还可见不同程度的口腔粘膜损害,口腔粘膜损害以RDEB患者多见和严重。Objective ： To characterize the oral manifestations of Epidermolysis Builosa cases. Methods ： The clinical data from 36 cases collected and analyzed from the literatures reported in Chinese since 1990. Results： （1） The oral manifestations of EB range from relatively mild to extremely severe. Epidermolysis Bullosa cases presented with infrequent blistering and ulceration of the oral mucosa to significant oral blistering and severe mucosal involvement. （2） Mucosal involvement occurred most commonly in RDEB with about 66. 7% of all individuals with EB compared with other subtypes of EB. （3） Those patients existed differences in interfamilial and intrafamilial oral lesions of EB. Especially, RDEB individuals were severely affected and exhibited with severe mucosal involvement. Conclusions ： Epidermolysis Bullosa （ EB ） developing oral manifestations or complications as well as blistering and mechanical fragility of the skin. Individuals of RDEB tend to be with severe mucosal involvement.

关 键 词：大疱性表皮松解症 常染色体遗传 Ⅶ型胶原 口腔粘膜 

分 类 号：R758.59[医药卫生—皮肤病学与性病学]