Howel-Evans syndrome: a variant of ectodermal dysplasia.
Howel-Evans syndrome is a rare form of palmoplantar keratoderma associated with esophageal cancer and is inherited in an autosomal dominant fashion. First described in 2 kindreds in the United Kingdom, Howel-Evans syndrome has subsequently been reported in only one American family. We present a previously unreported case of Howel-Evans syndrome from this American kindred demonstrating a distinct clinical phenotype. The patient manifests both cutaneous and ectodermal abnormalities, supporting the reclassification of Howel-Evans syndrome as a variant of ectodermal dysplasia.
Sroa
Novie
N
The Ohio State University, Division of Dermatology, Columbus 43221, USA. novie.sroa@osumc.edu
Witman
Patricia
P
D002648:Child; D004476:Ectodermal Dysplasia / Q000235:genetics / Q000503:physiopathology*; D004938:Esophageal Neoplasms / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000235:genetics / Q000503:physiopathology*; D008297:Male; D010641:Phenotype; D013577:Syndrome