Aplasia cutis congenita: Report of four cases and literature review

Aplasia cutis congenita (ACC) is a defect of cutaneous development, most commonly on the scalp. The incidence is between 0.5-1/10,000 of newborns. It can be associated with other congenital syndromic anomalies. Different aetiological theories exist about ACC. The defects are probably not attributable to one single factor. Management of the less severe forms is relatively simple and treatment can be either conservative or operative. More severe cases involving the skull are associated with a 20-55% mortality rate, due to massive haemorrhage or infection. Four new cases are reported; three were treated conservatively, one patient had a severe form which was associated with other congenital anomalies and died 1 month after birth. This small series confirms the effectiveness of conservative therapy in the treatment of limited areas of ACC. © Springer-Verlag 2004.