S4570 A Rare Case of Cronkhite-Canada Syndrome in a Young Male: Diagnostic and Therapeutic Challenges

Introduction: Cronkhite–Canada Syndrome (CCS) is a rare, nonhereditary gastrointestinal polyposis disorder characterized by chronic diarrhea, weight loss, and ectodermal changes such as alopecia and hyperpigmentation. Its pathogenesis remains unclear, and diagnosis relies on clinical, endoscopic, and histological findings. Case Description/Methods: A 38-year-old man presented with a 2-month history of postprandial diarrhea, nausea, vomiting, and generalized weakness, along with a 15 kg unintentional weight loss. He reported chronic abdominal pain worsened by meals and occasional blood-streaked stools, without melena or hematemesis. Physical examination revealed signs of malnutrition and ectodermal changes, including alopecia, nail dystrophy, and hyperpigmentation of the palms and soles. Laboratory workup showed hypoalbuminemia and iron deficiency anemia. Infectious screening, including stool cultures, Clostridioides difficile toxin, ova and parasite testing, and viral panels, was negative. Endoscopic evaluation revealed erythematous mucosa in the stomach, duodenum, terminal ileum, and colon. Biopsies showed polypoid mucosa with cystically dilated glands, edematous lamina propria, and mixed inflammatory infiltrates rich in eosinophils and mononuclear cells, without dysplasia. Video capsule endoscopy demonstrated diffusely edematous and nodular gastric mucosa, elongated villi throughout the small bowel, and dark blood in the proximal ileum without a clear bleeding source. These findings supported a diagnosis of Cronkhite–Canada Syndrome. The patient was started on a short course of intravenous corticosteroids, followed by oral prednisolone and azathioprine (Imuran) as a steroid-sparing agent. He also received nutritional support, proton pump inhibitors, and pancreatic enzyme supplements. At follow-up, the patient reported marked improvement in diarrhea, suggesting a positive response to immunosuppressive therapy. He remains under surveillance with routine labs and a scheduled endoscopy to assess mucosal healing. Discussion: This case underscores the importance of early diagnosis and multidisciplinary care in managing Cronkhite–Canada Syndrome, a rare but serious gastrointestinal polyposis disorder. The patient’s clinical improvement on steroids and azathioprine highlights the role of immunosuppression in addressing the inflammatory nature of CCS. Prompt treatment, nutritional support, and close surveillance are key to improving outcomes and reducing the risk of complications such as malnutrition and malignancy.