Ectodermal dysplasias associated with clefting: Significance of scalp dermatitis

Scott W. Fosko, Kurt S. Stenn, Jean L. Bolognia

Research output: Contribution to journalArticle

60 Scopus citations

Abstract

Several clinical syndromes are characterized by ectodermal dysplasia (ED) in association with clefting of the lip and/or palate. The three most commonly recognized entities are (1) the EEC syndrome (ectodermal dysplasia, ectrodactyly, cleft lip/palate); (2) the Rapp-Hodgkin syndrome with ectodermal dysplasia, cleft lip/palate, and mid facial hypoplasia; and (3) the Hay-Wells or AEC syndrome (ankyloblepharon, ectodermal defects, cleft lip/palate). The clinical characteristics of these entities as well as several less common syndromes are reviewed and summarized. The presence of scalp dermatitis in patients with the AEC syndrome and less often the Rapp-Hodgkin syndrome is emphasized.

Original languageEnglish (US)
Pages (from-to)249-256
Number of pages8
JournalJournal of the American Academy of Dermatology
Volume27
Issue number2
DOIs
StatePublished - Jan 1 1992

ASJC Scopus subject areas

  • Dermatology

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