Vanderhooft S L, Stephan M J, Sybert V P
Department of Medicine (Dermatology), University of Washington School of Medicine, Seattle 98195.
Pediatr Dermatol. 1993 Dec;10(4):334-40. doi: 10.1111/j.1525-1470.1993.tb00394.x.
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.
海-韦综合征是一种常染色体显性疾病，其特征为先天性丝状粘连性睑缘粘连、外胚层发育不良以及伴有或不伴有唇裂的腭裂（AEC综合征）。尽管有报道称部分患者出生时皮肤糜烂且头皮反复感染，但这些通常不被视为该疾病的主要特征。根据我们的经验，出生时皮肤裸露以及伴有感染的慢性头皮糜烂是该综合征的常见特征。建议积极进行伤口护理并尽早局部或全身应用抗生素。
