Yen S L, Chao L H, Ho M M, Hwang K C
Department of Pediatrics, Taiwan Provincial Tao-Yuan General Hospital, R.O.C.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1990 Nov-Dec;31(6):383-7.
Epidermolysis bullosa (EB) is a group of inherited skin diseases manifested as bullae formation induced by minor trauma. According to the anatomical level of skin splitting, it could be classified mainly into three types: simplex, junctional and dystrophic EB. We have a female term newborn noted to have had skin defect and ulceration over lower legs concurrent with oral mucosal ulceration and blister formation since birth. New blisters occurred over friction and pressure areas shortly after birth. The histopathologic pictures revealed dystrophic type of EB. These lesions seemed responsive to phenytoin and topical skin care, healing gradually with minimal scarring and atrophy. However, it is important to follow these patients to help alleviate complications of extracutaneous involvement which may occur during later life.
大疱性表皮松解症(EB)是一组遗传性皮肤病,表现为轻微创伤引起的水疱形成。根据皮肤裂开的解剖层面,它主要可分为三种类型:单纯型、交界型和营养不良型EB。我们有一名足月女婴,自出生以来小腿部存在皮肤缺损和溃疡,同时伴有口腔黏膜溃疡和水疱形成。出生后不久,摩擦和受压部位出现新的水疱。组织病理学图片显示为营养不良型EB。这些病变似乎对苯妥英和局部皮肤护理有反应,逐渐愈合,瘢痕形成和萎缩最小。然而,对这些患者进行随访很重要,以帮助减轻后期可能出现的皮肤外受累并发症。