Abdominal Aplasia Cutis Congenita with Fetus Papyraceus: Frieden’s Class V

Abstract

Aplasia cutis congenita (ACC) is a rare congenital disorder, characterized by the absence of the skin and adjacent tissue, usually affecting the scalp but can affect any part of the body. It may present as an isolated defect, accompanied by other developmental anomalies or syndrome. The present report describes the case of a 1-day-old full-term male child born with the absence of the skin over the abdomen which is a rare site for developmental defect in this disease. Antenatal ultrasonography findings in mother were suggestive of a twin pregnancy with one of the fetus presenting as fetus papyraceus (FP) classifying the present case under Frieden class V. Although there are many surgical techniques to manage ACC, the present case was managed conservatively and responded well. Type V ACC is unique in that the lesions are symmetric and distributed primarily on trunk. The involvement of upper and lower extremities may or may not be there. Although there is no well-defined therapeutic protocol from the available literature, conservative management is usually recommended. Early surgical intervention does not hold any added benefit.