Hintner H, Wolff K
Arch Dermatol. 1982 Jun;118(6):375-84.
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.
报告了8例遗传性大疱性表皮松解症（EB）的一种新变体——泛发性萎缩性良性EB。这是一种交界型EB，与赫利茨致死性EB不同，其预后良好。它以常染色体隐性性状遗传，在目前观察到的所有患者中临床表现单调相似，有泛发性水疱形成、萎缩性脱发和甲营养不良性改变。皮肤和黏膜上的水疱愈合后无瘢痕或营养不良，但常导致明显萎缩。随着患者年龄增长，症状有明确的改善趋势，但迄今为止治疗无效。
