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Adams-Oliver syndrome (AOS) is a congenital condition characterized by congenital aplasia cutis and transverse limb defects. Herein we report a case of an infant with severe intra-uterine growth restriction presenting AOS associated with cutis marmorata telangiectatica but with no other organ complications. The outcome was complicated by hemorrhagic and septic shock, which resulted in the death of the infant in a setting of multiorgan failure., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Introduction: Congenital atrichia with papular lesions is a rare congenital ectodermic dysplasia., Case Report: A male child had atrichia of the scalp and eyebrows from birth. The atrichia was associated with epidermic cystic lesions of the scalp. Biopsy showed cystic piler follicles within the derma surrounded with an inflammatory infiltration of multinucleated giant cells., Discussion: In most reported cases, scalp hair is present at birth and then falls within the first three months of life. Follicular cysts appear later. This syndrome generally occurs sporadically and the mode of inheritance is unknown.