Severe skin erosions and scalp infections in AEC syndrome.
Hay-Wells syndrome is an autosomal dominant condition characterized by ankyloblepharon 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 of 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.
Vanderhooft
S L
SL
Department of Medicine (Dermatology), University of Washington School of Medicine, Seattle 98195.
Stephan
M J
MJ
Sybert
V P
VP
D000015:Abnormalities, Multiple; D002648:Child; D002971:Cleft Lip; D002972:Cleft Palate; D004476:Ectodermal Dysplasia / Q000473:pathology*; D005143:Eyelids / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012535:Scalp / Q000473:pathology; D012536:Scalp Dermatoses / Q000150:complications* / Q000473:pathology; D013207:Staphylococcal Skin Infections / Q000150:complications*; D013577:Syndrome