Reichert S, Cocciale M P, Lemelle J L, Barbaud A, Schmitt M, Weber M, Schmutz J L
Service de Dermatologie, Hôpital Fournier, Nancy.
Ann Dermatol Venereol. 1997;124(6-7):470-3.
Congenital skin defect is an uncommon condition. The term of "congenital skin aplasia" should be avoided since the origin may not necessarily be congenital malformation. The scalp is involved in 80 p. 100 of the cases.
We observed a congenital skin defect located exclusively on the trunk. The infant lacked wide areas of skin in symmetrical star-like configurations.
The notion of a twin expulsed after 4 months gestation suggested the diagnosis of congenital skin defect and fetus papyraceus or group V congenital skin aplasia in the Frieden classification as recalled. This diagnosis must not be overlooked as the prognosis is good. Closure with atrophic skin usually occurs within a few weeks. The etiopathogenesis remains obscure.
先天性皮肤缺损是一种罕见的病症。应避免使用“先天性皮肤发育不全”这一术语，因为其病因不一定是先天性畸形。80%的病例累及头皮。
我们观察到一例仅位于躯干的先天性皮肤缺损。该婴儿有对称的星状大片皮肤缺失区域。
妊娠4个月后娩出双胎的情况提示诊断为先天性皮肤缺损，以及如前所述的弗里登分类中的纸样胎儿或V型先天性皮肤发育不全。由于预后良好，该诊断不容忽视。通常在几周内萎缩性皮肤会愈合。病因仍不清楚。
