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Severe Skin Erosions and Scalp Infections in AEC Syndrome
Author(s) -
Vanderhooft Sheryll L.,
Stephan Mark J.,
Sybert Virginia P.
Publication year - 1993
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1993.tb00394.x
Subject(s) - medicine , scalp , dermatology , ectodermal dysplasia , scarring alopecia , antibiotics , surgery , microbiology and biotechnology , biology
Hay‐Wells syndrome is an autosomal dominant condition characterized by ankytoblepharon filiforme adnatum, ectodermal dysplasia, and cleft palate with or without associated cleft lip (AEC syndrome). Although several reported patients had eroded skin at birth and recurrent scalp infections, these are not generally regarded as major features ot the disorder. In our experience, denuded skin at birth and chronic scalp erosions complicated by infection are common features of this syndrome. Aggressive wound care in conjunction with early administration of topical or systemic antibiotics is suggested.