Cronkhite-Canada Syndrome

aA 78-year-old woman previously diagnosed with irritable bowel syndrome presented with 6 months of progressive abdominal pain, gastroesophageal reflux, loss of taste, diarrhea (up to 10 episodes daily), intermittent bright red blood per rectum, and a 15-kg weight loss. Physical examination revealed onychodystrophy. Subsequent esophagogastroduodenoscopy showed multiple hyperplastic polyps in the stomach (Figure A) and complete replacement of the antrum of the stomach with polyps (Figure B). Colonoscopy revealed diffuse areas of altered vascular, congested, nodular mucosa with innumerous inflammatory and adenomatous polyps of varying sizes (Figures C and D). Capsule endoscopy also demonstrated multiple polyps throughout the entire small intestine. Given the clinical history, the patient was diagnosed with Cronkhite-Canada syndrome and treated with a 6-week taper of high-dose prednisone followed by budesonide, 9 mg daily (1 open capsule, 2 closed capsules), in conjunction with intermittent courses of rifaximin with complete resolution of her symptoms. Cronkhite-Canada syndrome is an extremely rare, noninherited condition associated with widespread gastrointestinal polyps, alopecia, onychodystrophy, skin hyperpigmentation, and diarrhea. Because of its rarity, the etiology, pathophysiology, and optimal treatment is not well established. Prior reports have found success in treatment with nutritional supplementation and immunosuppressive therapy, suggesting an underlying immune-mediated mechanism.