Generalized atrophic benign epidermolysis bullosa.
Generalized atrophic benign epidermolysis bullosa.
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DOI：
10.1001/archderm.1982.01650180009008
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发表时间：
1982-06
影响因子：
--
通讯作者：
T. Darling;Bauer Jw;H. Hintner;K. Yancey
中科院分区：
文献类型：
--
作者：
T. Darling;Bauer Jw;H. Hintner;K. Yancey
本文报告遗传性大疱性表皮松解症的一种新变种--泛发性萎缩性良性大疱性表皮松解症。这是一种交界型EB，与Herlitz的EB相比，预后良好。它是一种常染色体隐性遗传特征，目前观察到的所有患者的临床表现都是单调相似的，有泛发性水泡形成、萎缩性脱发和营养不良的指甲改变。皮肤和粘膜上的水泡愈合时没有疤痕或营养不良，但通常会导致明显的萎缩。随着患者年龄的增长，症状有一定的改善趋势，但到目前为止，治疗还没有效果。
Eight cases of a new variant of hereditary epidermolysis bullosa (EB), generalized atrophic benign EB, are reported. This is a junctional form of EB that, in contrast to EB letalis of Herlitz, has a good prognosis. It is inherited as an autosomal recessive trait, and the clinical picture is monotonously similar in all patients observed so far, with generalized blister formation, atrophic alopecia, and dystrophic nail changes. Blisters on the skin and mucous membranes heal without scarring or dystrophy but often result in notable atrophy. There is a definite tendency for amelioration of symptoms as the patients age, but therapy has, so far, been ineffective.