Aplasia cutis congenita. A follow-up evaluation after 25 years

Abstract

Aplasia cutis congenita is a rare disease with less than 300 cases reported in the literature. A patient with extensive involvement of the skin was observed for 25 years. During adolescence, a reactive depression related to the residual cicatricial alopecia on the left side of the scalp developed. The psychiatric distress disappeared after corrective surgery was performed. It is recommended that patients with this disorder be regularly observed to select cases appropriate for early plastic surgery.

Duodenal Atresia, Biliary Atresia, and Intestinal Infarct in Truncal Aplasia Cutis Congenita

“…Incomplete closure of the neural tube is suggested to explain midline lesions, but this theory does not account for truncal or limb defects (3). The vascular theory suggests that there is vascular insufficiency to the skin, perhaps from placental compromise or thromboplastic material from a fetus papyraceus (6–8). The amniogenic theory suggests that amniotic membrane adhered to fetal skin leads to ACC (9).…”

Aplasia Cutis Congenita: A Report of 12 New Families and Review of the Literature

“…Aplasia cutis congenita (ACC) was first described more than 150 years ago by Campbell (1), who reported two siblings with absence of the skin on the vertex of the scalp. Since that report, there have been numerous publications of sporadic and familial instances of classic ACC of the scalp , ACC involving the body (4,20,25,27,37,39,(43)(44)(45)(46)(47)(48)(49)(50)(51)(52)(53), in association with limb defects (12,15,17,27,29,39,(54)(55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65)(66), and with epidermolysis buUosa (67)(68)(69)(70)(71)(72)(73)(74)(75)(76)(77)(78)(79)(80)…”

Scalp Aplasia Cutis Congenita Presenting With Sagittal Sinus Hemorrhage

“…[15][16][17][18][19][20] If the defect involves the skull, repairing it with a skin graft can be problematic, because the direct application of splitthickness skin to the dura may preclude or retard spontaneous closure of the bony defect. 7,17,21,22 The growing brain will exert expanding forces on the graft, which may lead to graft atrophy or breakdown. Subsequently, acrocephaly may result owing to delayed or failed ossification of the fontanels.…”