Cronkhite Canada syndrome: case report with some features not previously described

Abstract

A 69-year-old woman was admitted to our unit with a history of diarrhea and weight loss lasting 1 year. Diarrhea consisted of 10 to 15 bowel movements per day without either blood or mucus. No abdominal pain was present. Her weight had fallen from 100 kg to 70 kg the year prior to examination. She also noted gradual loss of hair and increasing thinness and brittleness of fingernails and toenails. On examination, the patient appeared to be overweight and dehydrated. She presented irregular alopecia and hair fell out upon being touched. There was brown pigmentation on the back of her hands and in the periorbital region, while the fingernails and toenails were atrophied with onychodystrophy. The remainder of the physical examination was unremarkable. Serum laboratory values were: white blood count 3100, hemoglobin 11 g, hematocrit 29%, and iron 49 /lgjdl. Electrolytes were: sodium 136 mmol/liter, and potassium 2.4 mmol/liter. Total proteins were 6 gjdl and albumin was 2.7 gjdl. Gastrin was 1000 pgjml (NV, 20 to 80) and there was no gastric acid production (MAO, 0); anti-gastric parietal cell antibody test was positive. Fecal microscopy and culture were negative for pathogens. Liver and kidney function tests were normal. Evaluation of endocrine function was normal. Secretion test of pancreatic function showed normal volume and bicarbonate output. Double-contrast barium enema and x-rays of the upper gastrointestinal tract revealed multiple filling defects of the colon and stomach. A small bowel enema did not show any polypoid lesions. The upper gastrointestinal endoscopy showed about 10 (0.5 to 3 cm) sessile polyps in the stomach (Fig. 1); the esophagus and the duodenum were free ofpolyps. The gastric mucosa between polyps was thin and pale, without erosions or ulcers. Colonoscopy revealed multiple (about 20) sessile and pedunculated polyps distributed throughout the colon. Histologic analysis of the gastric polyps showed the surface epithelium to be mostly flattened without ulceration