IBD in Camouflage or Are There Similarities?: A Case of Cronkhite-Canada Syndrome

First described in 1955, Cronkhite Canada Syndrome (CCS) is a rare non-familial polyposis syndrome with approximately 500 cases to date. With a five-year mortality of 50%, it is crucial to correctly and quickly identify the syndrome's primary features of alopecia, onychodystrophy, cutaneous hyperpigmentation, and intestinal malabsorption. Unfortunately, even when diagnosed, treatment is challenging as the etiology is unknown though possibly immune-mediated. Antibiotics, steroids, and immunosuppressants have all shown varying degrees of success. A 74-year-old Filipino woman with a history of hypothyroidism presented to our Inflammatory Bowel Disease (IBD) Clinic for a second opinion. Seven months prior, she experienced onset of watery, non-bloody diarrhea with initial diagnosis of ulcerative colitis. After this diagnosis, she responded to treatment with prednisone, but failed maintenance therapy with oral mesalamine. She then received two doses of infliximab without response and was referred to our IBD clinic. She reported continued watery, non-bloody diarrhea with significant weight loss. Physical exam revealed alopecia, onychodystrophy, and lower extremity edema with cutaneous hyperpigmentation. Her labs revealed hyponatremia, severe hypoalbuminemia, c-reactive protein (CRP) of 55.3 mg/L (0-8.2 mg/L), and a hemoglobin of 10.1 g/dL which prompted hospital admission.Inpatient EGD and colonoscopy revealed diffuse polyposis throughout the stomach, duodenum, and colon. Biopsies revealed diffuse inflammatory hyperplastic polyps with increased IgG4 positive cells (up to 22 per high power field). Based on this clinical picture, she was diagnosed with CCS. She was placed on full bowel rest and given total parenteral nutrition. She also received IV corticosteroids in conjunction with azathioprine and infliximab. We achieved good clinical response and her CRP normalized. CCS is a rare non-familial polyposis syndrome with characteristic physical exam findings, diffuse polyposis, debilitating diarrhea, and significant 5-year mortality. Treatment is challenging and focuses primarily on nutritional support and immunosuppressant medications. In this particular case we chose to proceed with low dose azathioprine and infliximab based on success of this regimen in past case reports published. We will plan to achieve steroid free remission by optimizing infliximab based on levels and providing nutritional support.2073_A Figure 1. Onychodystrophy2073_B Figure 2. Diffuse polyposis on colonoscopy2073_C Figure 3. Diffuse inflammatory hyperplastic polyps with increased IgG4 cells