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.