Diffuse gastrointestinal polyposis with ectodermal changes.

Hereditary polyposis of various parts of the alimentary tract has been well recognized for several decades and, in a recent review, McKusick (1962) described six distinct varieties. On the other hand only three cases of diffuse gastrointestinal polyposis, associated with ectodermal changes, have been described. These patients had no family history. They presented with diarrhoea, atrophy of the nails, pigmentation of the skin, and alopecia. They were found to have polyposis involving the stomach, small intestine, and colon and died from the disease a few months after the diagnosis was made (Cronkhite and Canada, 1955: Johnston, Vosburgh, Wiens, and Walsh, 1962). It seems likely that such cases belong to a distinct syndrome in view of the lack of family history, and the peculiar associated ectodermal changes. Recently we had the opportunity to study a patient with the characteristics of the syndrome described by Cronkhite and Canada, who had polypoid mucosa from the cardia to the rectum, and whose condition improved dramatically after partial gastrectomy and replacement therapy.