Large scalp and skull defect in patient with aplasia cutis congenita

Aplasia cutis congenita is a rare condition characterized by the absence of skin layers. It is most common on the scalp, middle line, and it can be seen as a congenital ulcer involving periosteum, skull and dura. We present the case of a female newborn infant with a dysmorphic facies, a large scalp...

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Bibliographic Details
Published in:	Arquivos de neuro-psiquiatria Vol. 62; no. 4; pp. 1108 - 1111
Main Authors:	Henriques, José Gilberto de Brito, Pianetti Filho, Geraldo, Giannetti, Alexandre Varella, Henriques, Karina Santos Wandeck
Format:	Journal Article
Language:	English Portuguese
Published:	Brazil 01-12-2004
Subjects:	Ectodermal Dysplasia - pathology Ectodermal Dysplasia - therapy Encephalocele - etiology Encephalocele - pathology Fatal Outcome Female Humans Infant, Newborn Scalp - abnormalities Skull - abnormalities
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Summary:	Aplasia cutis congenita is a rare condition characterized by the absence of skin layers. It is most common on the scalp, middle line, and it can be seen as a congenital ulcer involving periosteum, skull and dura. We present the case of a female newborn infant with a dysmorphic facies, a large scalp and skull defect exposing the dura. There was no cerebrospinal fluid leakage. The rarity of cases with large defects and small series reported make difficult to determinate the ideal treatment for aplasia cutis congenita. More studies are necessary to define the etiology and best management of this patients.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0004-282X
DOI:	10.1590/S0004-282X2004000600034