Type V aplasia cutis congenita with fetus papyraceus

Abstract

Aplasia cutis congenita (ACC) is a condition characterized by the congenital absence of all skin layers in a localized or widespread area. This phenomenon was first described by Cordon in 1767.1 More than 2 centuries later, Frieden2 proposed a classification system for clinical subtypes of ACC based on the affected area(s), associated anomalies, and mode of inheritance. This categorization recognized a rare subtype of ACC that is associated with multiple gestation in which there is the in utero demise of a twin with resultant fetus papyraceus (FP), or mummification. FP is thought to develop from compression of the expired twin by the viable fetus as it grows, causing a phenomenon known as vanishing twin syndrome. Frieden2 designated this subset of cases as type V ACC. ACC with FP is clinically unique in that it is characterized by stellate lesions in a symmetrical distribution over the trunk and extremities, differing from other subtypes of ACC, which are typically localized to the scalp (70%-85% of cases) or extremities. Apart from isolated scalp involvement, there are little epidemiologic data delineating the frequency of other distribution patterns. Here we present a case of type V ACC associated with FP.