Congenital skin defects and fetus papyraceus

Two unrelated infants with congenital skin defects (aplasia cutis congenita) involving the trunk and limbs, each with an associated monozygotic twin fetus papyraceus, are described. Evidence from these two cases and a review of the literature indicate that congenital skin defects of other body areas, as represented by these two children, is a specific pattern of malformation distinct from isolated small congenital skin defects involving the vertex of the scalp. The frequent occurrence of fetus papyraceus in patients with congenital skin defects of other body areas suggests a common etiology for these two phenomena.