全消化管を検索し得たCronkhite-Canada症候群の1例

抄録

A 60-year-old woman was referred to our hospital with nausea, vomiting, taste disorder, alopecia, onichodystrophy and epigastric pain. Laboratory examination showed under-nutritionand abdominal computed tomography showed a thickened stomach wall. Endoscopic examination of the entire digestive tract showed multiple salmon roe-like polyp in the stomach, duodenum, ileum, and colon. Pathologically, biopsy specimens from the polyp-like lesions and mucosa revealed edematous change of the intestitium and cystic dilatation of the glands. We diagnosed the patient as having Cronkhite-Canada syndrome because of the polyposis associated with abnormalities of the ectoderm.
Prednisolone therapy was initiated at 40 mg per day, with tapering of the dose subsequently. The clinical symptoms, laboratory data and polyposis improved with the prednisolone therapy. The prognosis of patients with Cronkhite-Canada syndrome treated with prednisolone is favorable, however, it has been reported recently that Cronkhite-Canada syndrome is associated with a risk of carcinogenesis. Therefore, it is necessary to conduct periodic screening of the entire digestive tract even after mucosal improvement.