“…These vascular disruptions especially in Bwatershed[ areas such as the cranial vertex and limbs result in ischemia and are thought to be sufficient to result in ACC. 2,6,15,17,18 Aplasia cutis congenita usually presents as a solitary defect but can be associated with many different structural abnormalities including cleft lip and palate, limb reduction defects, epidermolysis bullosa, duodenal/ileal atresia, patent ductus arteriosus, omphalocele, meningocele and encephalocele, polycystic kidney, gastroschisis and neurologic malformations, ectodermal dysplasia, and fetus papyraceus. 7,15,16 The infants with fetus papyraceus tend to have large cutaneous truncal and limb defects, with the scalp often spared.…”