Cronkhite-Canada Syndrome in an African American Male

Background: Cronkhite-Canada Syndrome (CCS) is an rare disorder characterized by protein-losing enteropathy, ectodermal changes including alopecia, hyperpigmentation, onychodystrophy, and hamartomatous polyps in the GI tract. CCS is a non-inherited condition that is often fatal. A few hundred cases have been identified worldwide since the discovery of the disease in 1955, with approximately 75%of the case reports appearing in the Japanese population. Here we report a case of CCS in an African American male. Case: A 64 year-old African American male with a history of hypertension, Hepatitis C virus, Degenerative Joint Disease, and Hyperlipidemia presented to the Emergency Department after a presyncopal episode. The patient had undergone spinal fusion surgery of the L2-L3, L3-L4, and L4-L5 vertebrae, one month prior to admission. The surgery was complicated by a prolonged period of anesthesia. Immediately after surgery he developed persistent diarrhea, approximately 5 episodes per day, and a 30-pound weight loss. He also reported new onset alopecia and skin discoloration. He denied nausea, vomiting, abdominal pain, fever, or chills. Clinical / Laboratory Findings: Physical exam was significant for orthostatic hypotension, diffuse alopecia and hyperpigmentation of the face and palms (Figure. 1 - L), and dystrophic nail changes (Figure. 1 - R).Figure 1He had a soft, non-tender abdomen and no lymphadenopathy. - Labs were significant for low potassium (2.9) and albumin (3.1) and elevated ESR (33) and CRP (6.1). - Stool studies including Clostridium difficile, fecal leukocytes, and ova and parasites, were negative. - 2D ECHO was unremarkable - Pan-CT scans of the head, chest, abdomen, and pelvis were unrevealing for any potential etiology of the patient's presentation.Figure 2Discussion: This clinical vignette presents an extremely rare case of Cronkhite-Canada Syndrome in an African American male. Most reported cases are in the Japanese population, with a male to female ratio of 2:1. Little is known about the etiology of this severe protein-losing enteropathy. Histologically, the most prominent features of CCS are edema of the lamina propria in the mucosa of the gastrointestinal tract and eosinophilia (Figure 3-4). Unfortunately, the prognosis for this syndrome is poor, with a 55% 5-year mortality. No evidenced based therapies are available, given the rarity of the disease, but hyperalimentation and corticosteroids are mainstays of treatment. More recently immunomodulatory therapy with azathioprine has shown some efficacy in treating this disorder.Figure 3

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