Aplasia cutis congenita--a case study of a family with two siblings with this disorder.

ABSTRACT

BACKGROUND: Aplasia cutis congenita (ACC) is a rare dermatologic disorder. The cause is unknown. It is a heterogeneous group of disorders whose common characteristic is focal absence of skin. It is a congenital absence of epidermis. In the majority of cases, this is limited to the scalp, although other areas may also be involved. The lesions are non-inflammatory and well demarcated. These lesions can be circular, linear, oval or stellate and range in size from 0.5 to 10 cm. Treatment has been controversial.