Kanzler M H, Smoller B, Woodley D T
Division of Dermatology, Santa Clara Valley Medical Center, San Jose, Calif. 95128.
Arch Dermatol. 1992 Aug;128(8):1087-90.
Congenital localized absence of the skin has been observed in various subsets of inherited epidermolysis bullosa. Through electron microscopy and immunomapping, we attempt to clarify the relationship of congenital localized absence of the skin lesions to epidermolysis bullosa.
The case of a child with epidermolysis bullosa simplex and congenital localized absence of the skin is presented. Electron microscopy and immunomapping of the areas of congenital localized absence of the skin and sites of skin fragility suggest that these lesions are pathogenically identical.
After reviewing the literature, we believe that the term Bart's syndrome should be used to identify patients with any type of epidermolysis bullosa who present with localized congenital absence of the skin on the extremities.
在遗传性大疱性表皮松解症的不同亚组中观察到先天性局限性皮肤缺失。通过电子显微镜和免疫定位，我们试图阐明先天性局限性皮肤病变与大疱性表皮松解症之间的关系。
报告了1例单纯型大疱性表皮松解症合并先天性局限性皮肤缺失患儿的病例。对先天性局限性皮肤缺失区域和皮肤脆弱部位进行电子显微镜检查和免疫定位，结果表明这些病变在发病机制上是相同的。
在复习文献后，我们认为“巴特综合征”这一术语应用于识别任何类型的大疱性表皮松解症且伴有四肢先天性局限性皮肤缺失的患者。
