Cronkhite–Canada Syndrome

Synonyms

CCS

Definition

Cronkhite–Canada syndrome (CCS) was first described in 1955 by Leonard W. Cronkhite and Wilma Jeanne Canada as a new distinct clinical entity, occurring in two patients with generalized gastrointestinal polyposis, pigmentation of the skin, alopecia, and atrophy of the fingernails and toenails.

Is a rare, non-inherited gastrointestinal polyposis syndrome characterized by diffuse distribution of hamartomatous or juvenile-type polyps throughout the gastrointestinal tract, except for the characteristic sparing of the esophagus and specific ectodermal abnormalities, including alopecia, onychodystrophy, and skin hyperpigmentation.

CCS is a chronic disorder of unknown etiology. An autoimmune origin has been suggested but not proved. Other proposal theories included an infectious cause, nutritional deficiency, altered intestinal mucin production, and mast cell dysfunction.

Diarrhea is the most prominent feature, and patients also can have weight loss, abdominal pain,...