Severe Skin Erosions and Scalp Infections in AEC Syndrome

Authors

  • Sheryll L. Vanderhooft M.D.,

    Corresponding author
    1. Department of Medicine (Dermatology), University of Washington School of Medicine, Seattle, Washington.
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  • Mark J. Stephan M.D.,

    1. Department of Pediatrics (Dysmorphology Unit), Madigan Army Medical Center, Tacoma, Washington.
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  • Virginia P. Sybert M.D.

    1. Departments of Pediatrics (Medical Genetics) and Medicine (Dermatology), University of Washington School of Medicine and Children's Hospital and Medical Center, Seattle, Washington.
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  • The opinions and assertions contained herein are the views of the authors and are not to be construed as official or as reflecting the views of the United States Department of the Army or the Department of Defense.

Address correspondence to Sheryll L. Vanderhooft, M.D., University of Washington, Division of Dermatology, RM-14, Se-attle, WA 98195.

Abstract

Abstract: 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.

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