A Very Unusual Cause of Diarrhea in a 60-Year-Old Man

Abstract

Purpose: A 60-year-old man presented with a nine-month history of diarrhea associated with 40-lb weight loss. He also complained of gradual loss of his nails and hair, burning of the tongue, decreased taste sensation and hyperpigmentation of his skin. He had a screening colonoscopy at the age of 50, which was normal. His laboratory tests were significant for low albumin and hemoglobin. Stool studies were all negative. A CT abdomen showed abnormal thickening of the distal gastric body and antrum. An upper endoscopy revealed diffuse mucosal abnormality characterized by erythema and nodularity in the gastric body and antrum, as well as in the duodenum. A colonoscopy revealed many small-to-medium sized polyps throughout the colon, along with patchy areas of moderately congested and nodular mucosa in the colon and the terminal ileum. Gastric biopsies revealed edema and foveolar hyperplasia suggestive of hyperplastic polyps, but no dysplasia. Random colonic biopsies revealed active focal colitis with multiple fragments of inflammatory polyps. The patient was diagnosed with Cronkhite-Canada syndrome (CCS). He was treated with prednisone and azathioprine with subsequent improvement in symptoms. CCS is an extremely rare non-hereditary disorder characterized by diffuse gastrointestinal polyps, diarrhea, weight loss, onychodystrophy, alopecia, hypoguesia and skin hyperpigmentation. Ever since being first described in 1955, a little over 400 cases have been reported worldwide with the majority of patients being from Japan. The GI tract polyps are of the hamartomas non-neoplastic type. Dysplastic changes can develop in these polyps, and the lifetime colorectal cancer risk is about 25%. Mortality rate, mostly related to the protein-losing enteropathy approaches 50% at 5 years from the time of diagnosis. No specifi c treatment has been standardized, but corticosteroids and azathioprine have been used with good results. Successfully treated patients should clear most of the inflammatory polyps, and any remaining polyps should be resected, as they are more likely to have an adenomatous component. Any dysplastic changes detected may warrant intestinal resection.Figure: Colon polyps.