Aplasia cutis congenita: an association with vanishing twin syndrome

Abstract

Aplasia cutis congenita (ACC) is an inborn skin defect, variably associated with absence of the underlying subcutaneous fat and bone. Frieden IJ proposed a classification system for ACC consisting of 9 groups according to lesion distribution and associated malformations. We report a child resembling ACC group 5, with extensive truncal and limb skin defects and fetus papyraceous, the so-called vanishing twin syndrome. According to our experience, the risk of developing ACC with fetus payraceous is unpredictable, although twin abortion occurred during the first weeks of gestation. This case emphasizes the importance of a team approach to patients affected by wide skin lesions, and the usefulness of a precise anamnesis in order to clarify the etiology of this rare but often dramatic disorder.