Giant Aplasia Cutis Congenita without Associated Anomalies

Abstract

Aplasia cutis congenita is a congenital condition in which skin, bone, and dura can be absent. The condition can present in isolation or with associated conditions such as limb anomalies or embryologic malformations. The majority of cases affect the scalp and are limited to the dermis and epidermis. Vertex aplasia cutis typically range in size from 0.5 to 3 cm. The rare larger scalp defects are prone to complications of hemorrhage and infection, and subsequently patients are at risk for death. For these reasons, surgical intervention for large defects may be required. We report the case of a 12-month-old Haitian boy who presented with aplasia cutis congenita of the scalp involving 10 cm of skin and 9 cm of underlying bone. There were no other associated anomalies.