Cronkhite-Canada syndrome

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. In this chapter, we provide a succinct overview of the history, etiology, risk factors, clinical manifestations, diagnosis, and management of CCS. The latest evidence on the pathogenesis of CCS, as well as novel treatment options, will be incorporated. We anticipate that this chapter will enhance the knowledge of clinicians on this rare condition, helping to facilitate an appropriate diagnosis and enhance the management of patients with CCS going forward.