Carcinoma of the esophagus and tylosis. A lethal genetic combination.
Hyperkeratosis of the palms and soles (tylosis) is an uncommon genetic disorder. A small number of English families, however, have been described in which it is associated with carcinoma of the esophagus. The current report is of the first American family described with this condition. Members of those families affected with tylosis have at least a 90% risk of esophageal carcinoma by age 65 years. The paired conditions have an autosomal dominant mode of transmission and probably are controlled at a single genetic locus. The actual pathologic state might be mediated through an increase in epidermal growth factor receptors in the abnormal tissues.
Ohio State University College of Medicine, Columbus.
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D002294:Carcinoma, Squamous Cell / Q000150:complications / Q000235:genetics*; D066246:ErbB Receptors / Q000378:metabolism; D004938:Esophageal Neoplasms / Q000150:complications / Q000235:genetics*; D005192:Family Health; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000150:complications / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D016211:Transforming Growth Factor alpha / Q000378:metabolism
D016211:Transforming Growth Factor alpha; D066246:ErbB Receptors