title: [Aplasia cutis congenita: Update and management].
abstract: Congenital skin aplasia, or aplasia cutis congenita (ACC) is a rare congenital disease. It is characterized by the absence of skin at birth, localized or widespread, of one or several areas. This condition commonly involve the scalp but can also involve more rarely the trunk or limbs. However it is most frequently an isolated disorder, it can be associated with other anomalies, such as the Adams-Oliver syndrome, the association with a fetus papyraceus or with an epidermolysis bullosa. Many hypothesis have been suggested: vascular, genetic, traumatic, pharmacological or an anomaly in the neural tube closure process, but the exact mechanism is still unknown. Morbidity and mortality of this malformation depends on the affected area and the size of the defect. The main risk is the infection, hemorrhage and thrombosis in the case of a scalp defect with an underlying bone defect, the exposure of the meninges and the superior sagittal sinus. The initial management of ACC will therefore involve several plastic surgery techniques, from more simple to more complex, using conservative wound care to flaps techniques. Other techniques can be performed later, in the management of ACC sequelae, such as skin expansion for scarring alopecia.
authors: Service de chirurgie plastique, reconstructrice et esthétique, hôpital Roger-Salengro, CHRU de Lille, rue Emile-Laine, 59037 Lille cedex, France. Electronic address: anissa.belkhou@gmail.com.
authors: A
authors: A
authors: Belkhou
authors: Chirurgie plastique reconstructrice et esthétique, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51100 Reims, France; EA 3801, université de Champagne-Ardenne, 51, rue Cognacq-Jay, 51100 Reims, France.
authors: C
authors: C
authors: François
authors: Service de chirurgie plastique, reconstructrice et esthétique, hôpital Roger-Salengro, CHRU de Lille, rue Emile-Laine, 59037 Lille cedex, France.
authors: Y
authors: Y
authors: Bennis
authors: Service de chirurgie plastique, reconstructrice et esthétique, hôpital Roger-Salengro, CHRU de Lille, rue Emile-Laine, 59037 Lille cedex, France.
authors: V
authors: V
authors: Duquennoy-Martinot
authors: Service de chirurgie plastique, reconstructrice et esthétique, hôpital Roger-Salengro, CHRU de Lille, rue Emile-Laine, 59037 Lille cedex, France.
authors: P
authors: P
authors: Guerreschi
mesh_terms: D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000145:classification / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D006801:Humans
keywords: Adams-Oliver syndrome; Aplasie cutanée; Child; Congenital; Congénital; Enfant; Fetus papyraceus; Fœtus papyracé; Skin aplasia; Syndrome d’Adams-Oliver