先天性皮肤缺损12例临床分析

先天性皮肤缺损（ＣＡＳ）是一种少见的出生时皮肤局限或广泛缺损的疾病，以一定部位的皮肤缺损为特征，伴随多种异常、若干异常综合征及遗传方式。报告１２例ＣＡＳ，均有典型的皮肤症状，其中４例伴有其他异常：大疱性表皮松解症，趾甲缺损，胫腓骨远端细小畸形及二胫骨长短不一。

Analysis of 12 cases of congenital absence of skin

Congenital absence of skin is a rare condition in which localized or widespread areas of skin are absent at birth. It is characterized by the location and pattern of skin absence. the presence of associated malformations and a number of malformation syndromes, and the mode of inheritance. Clinical data of 12 affected newborn infants were analysed.All the patients had typical skin lesions, four of whom were associated with other malformations: epidermolysis bullosa, nails absence, slight distal tibioperoneal abnormalities,and different length of tibiae.