Guinard D, Lebeau J, Moutet F, Raphael B
Service de Chirurgie Plastique, Hôpital A. Michallon, CHRU, Grenoble.
Ann Chir Plast Esthet. 1993 Oct;38(5):513-9.
Aplasia cutis congenita is a rare malformation characterized by absence of skin. We report three new cases, localized on the vertex with one appearing as a familial form and requiring an emergent surgical repair with a favorable outcome. After a review of the literature, the authors discuss nosological aspects and etiological problems which are still not resolved. It seems that vascular differentiation of the skin could play an associated role. As regards of treatment, different attitudes are possible and are exposed, with a preference for surgery which is the method of choice for the authors.
先天性皮肤发育不全是一种以皮肤缺失为特征的罕见畸形。我们报告了3例新病例，均位于头顶，其中1例表现为家族性形式，需要紧急手术修复，结果良好。在回顾文献后，作者讨论了仍未解决的疾病分类学方面和病因问题。皮肤的血管分化似乎可能起相关作用。关于治疗，有不同的处理方式并进行了阐述，作者更倾向于手术，这是他们选择的治疗方法。
