Aplasia cutis congenita of extremities (group VII): case report from North-Western Nigeria

Abstract

Aplasia cutis congenita (ACC) is a rare congenital disorder characterised by localised or widespread absence of skin mainly affecting the scalp. Bilateral involvement of both the upper and lower extremities is uncommon. This is a case report of a rare congenital disorder. The patient was a 26-hour-old male baby admitted with extensive absence of skin on the lower and upper extremities. He was co-managed conservatively with the plastic surgical team for ACC group VII. The lesions were healing satisfactorily until 12 days into the admission when the parents signed against medical advice despite counselling. ACC with involvement of both upper and lower extremities is a rare presentation that responds to conservative treatment. The report emphasises the need for a legal framework for physicians to override the decision of the caregiver not in the best interest of a child.

Keywords: Aplasia cutis congenital; Extremities; Group VII; Nigeria.

Figure 1.

ACC. There is bilaterally symmetrical absence of skin over the anteromedial aspect of the lower extremities, the distal third of the arms extending to the proximal third of the forearms, the dorsal of the hands, and the distal third of the left forearm.