Diagnosis of a rare Syndrome: Cronkhite-Canada Syndrome

Abstract

Cronkhite-Canada syndrome (CCS) is a rare, non-familial disorder of unknown etiology associated with alopecia, cutaneous hyperpigmentation, gastrointestinal polyposis, onychodystrophy, diarrhea, weight loss and abdominal pain.The prevalence of gastrointestinal malignancy in CCS patients is about 13%, and especially is high in colorectal and gastric areas; 5 year mortality rate is 55%. In this report, a 74 year old man is described who had dysgeusia, skin hyperpigmentation, onycholysis, abdominal pain, chronic diarrhea, progressive weight loss and episodic melena since one year ago. He underwent upper endoscopy and colonoscopy. Diffuse polyposis were seen in stomach, duodenum and from rectum to cecum. Pathology of biopsy specimens showed hamartomatous polyps, compatible with Cronkhite-Canada syndrome.Although CCS is a rare acquired syndrome, it should be considered in differential diagnosis of gastrointestinal polyposis with diarrhea and skin changes. These patients need careful follow up to identify associated malignancies.