Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Cronkhite–Canada Syndrome

  • Andreia Albuquerque
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-40560-5_1451

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,...

This is a preview of subscription content, log in to check access.

References and Further Reading

  1. Cronkhite, L. W., Jr., & Canada, W. J. (1955). Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. The New England Journal of Medicine, 252, 1011–1015.PubMedCrossRefGoogle Scholar
  2. Daniel, E. S., Ludwig, S. L., Lewin, K. J., Ruprecht, R. M., Rajacich, G. M., & Schwabe, A. D. (1982). The Cronkhite-Canada Syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine (Baltimore), 61, 293–309.CrossRefGoogle Scholar
  3. Sweetser, S., Ahlquist, D. A., Osborn, N. K., et al. (2012). Clinicopathologic features and treatment outcomes in Cronkhite-Canada syndrome: support for autoimmunity. Digestive Diseases and Sciences, 57(2), 496–502.PubMedCrossRefGoogle Scholar
  4. Ward, E. M., & Wolfsen, H. C. (2003). Pharmacological management of Cronkhite-Canada syndrome. Expert Opinion on Pharmacotherapy, 4, 385–389.PubMedCrossRefGoogle Scholar
  5. Ward, E. M., & Wolfsen, H. C. (2002). Review article: The non-inherited gastrointestinal polyposis syndromes. Alimentary Pharmacology & Therapeutics, 16, 333–342.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Serviço de Gastrenterologia, Centro Hospitalar de São JoãoPortoPortugal