Cronkhite–Canada Syndrome

Abstract

Cronkhite–Canada syndrome (CCS) is a noninherited condition, associated with high morbidity, and characterized by gastrointestinal inflammatory polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea. The etiology of CCS is unknown, although evidence continues to emerge supporting an autoimmune basis. The diagnosis of CCS is clinicopathological and remains challenging. Corticosteroids and nutritional support remain the cornerstone of management, however, newer immunomodulatory agents have emerged as viable alternatives in recent years. The role of surgery remains limited to complications refractory to medical management. The question of whether polyps in CCS possess malignant potential remains controversial. Optimal cancer screening protocols have not been developed for CCS patients, owing to the rarity of the disease. However, careful surveillance is recommended.