S2277 An Unexpected Finding of Cronkhite-Canada Syndrome (CCS) Mimicking Inflammatory Bowel Disease

Introduction: Cronkhite-Canada Syndrome (CCS) is an extremely rare disease of unknown etiology, characterized by non-neoplastic diffuse polyposis, gastrointestinal symptoms, and ectodermal changes. Most cases have been reported in Japan, with an estimated incidence of one per million. We present a case of CCS in a Chinese patient presenting with diarrhea suspected to be secondary to inflammatory bowel disease (IBD) after failing outpatient oral corticosteroids. Case Description/Methods: A 54-year-old Chinese man, active smoker, with a history of chronic hepatitis B presented with complaints of progressively worsening weakness, generalized abdominal pain, non-bloody diarrhea, decreased oral intake and 10-pound weight loss over a 2-month period. He also observed cutaneous hyperpigmentation, brittle nails and hair loss. On physical examination, he was found to have visible emaciation, hyperpigmentation of bilateral hands and neck, grayish discoloration below the tongue and buccal mucosa, onycholysis, onychoschizia, finger clubbing, alopecia, madarosis, and bilateral 2+ pitting pedal edema. He was noted to have hypokalemia and hypoalbuminemia. Stool cultures grew Plesiomonas shigelloides (PS). CT scan of the abdomen and pelvis with intravenous (IV) contrast revealed thickened gastric folds, and distal ileal, pan-colonic and rectal nodular thickening, consistent with ileocolitis, suspicious for IBD. Subsequent outpatient upper endoscopy and colonoscopy revealed severe nodular pangastritis, proximal duodenitis, terminal ileitis, pancolitis and proctitis. Colonoscopy also revealed pseudo-polyps in a continuous and circumferential pattern from cecum to anus with histopathologic findings consistent with CCS. After a 2-week course of oral corticosteroids followed by azathioprine, his symptoms continued to progress, and he presented to the emergency department for evaluation. The patient was started on IV ciprofloxacin for PS and a 3-day course of IV corticosteroids, leading to improvement of diarrhea and facilitating discharge on oral prednisone with a close follow-up with gastroenterology (Figure 1). Discussion: Although etiology of CCS is not well understood, the disease has implications on quality of life with increase in morbidity and mortality. While CCS is more prevalent in the Asian population, clinicians should maintain a high level of suspicion, especially in a large tertiary care setting, catering to an ethnically diverse population. Further studies are needed to elicit underlying etiology and curative therapies.Figure 1.: A and B: Severe nodular pan-gastritis C: Severe nodular pan-duodenitis D: Terminal Ileitis E: Colitis of transverse colon F: Proctocolitis and pseudo polyps in a continuous and circumferential pattern G-I: The biopsy specimens from the upper and lower gastrointestinal tract show striking lamina propria edema in all specimens (G-I). In the stomach (G) and colon (I), the samples from both the polypoid and non-polypoid appearing regions had hyperplastic epithelium with cystically dilated glands. The duodenal sample picture here (H) demonstrates a dilated crypt with attenuated epithelium and luminal crypt abscess.

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