Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan

Abstract

One hundred and ten cases of Cronkhite-Canada Syndrome (C-C-S) reported in Japan were reviewed in this epidemiologic study. Seventy-five percent of all C-C-S cases reported in the world in literature have been reported from Japan. There has been no special occupation associated with an increased incidence of C-C-S. Mental and physical stress have been confirmed as among the most important risk factors for this syndrome. Hypogeusia is the dominant initial symptom which usually is followed by diarrhea and ectodermal changes including alopecia, nail dystrophy and skin pigmentation. Gastrointestinal polyposis is closely related to the malabsorption which induced these ectodermal changes. However, there is a small number of cases in which alopecia precedes to the diarrhea in the disease course.