2013
DOI: 10.1155/2013/856873
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Cronkhite-Canada Syndrome: Review of the Literature

Abstract: Cronkhite-Canada syndrome is a rare disease characterised by diffuse polyposis of the gastrointestinal tract, diarrhoea, weight loss, abdominal pain, cutaneous hyperpigmentation, dystrophic changes of fingernails, and alopecia. The etiology is probably autoimmune and diagnosis is based on history, physical examination, endoscopic findings of gastrointestinal polyposis, and histology. The disease is very rare; about 450 cases have been described in the literature so far. We present a review of the literature wi… Show more

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Cited by 55 publications
(72 citation statements)
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“…CCS is a rare disease, and only 450 cases have been described in the literature thus far since Cronkhite and Canada first reported two cases in 1955 (1,2). Approximately two-thirds of patients are of Japanese descent (3).…”
Section: Introductionmentioning
confidence: 99%
“…CCS is a rare disease, and only 450 cases have been described in the literature thus far since Cronkhite and Canada first reported two cases in 1955 (1,2). Approximately two-thirds of patients are of Japanese descent (3).…”
Section: Introductionmentioning
confidence: 99%
“…Differential diagnosis of CCS includes Menetrier disease, familial adenomatous polyposis, juvenile polyposis, Cowden syndrome, Peutz-Jeghers syndrome, inflammatory bowel disease, Whipple disease and small intestinal lymphoma (22)(23)(24). In the present case, the patient was initially diagnosed with familial polyposis, but eventually was diagnosed with CCS due to the dermatological triad of hyperpigmentation in oral mucosa (Fig.…”
Section: Discussionmentioning
confidence: 72%
“…Although there is no standardized treatment, current reports favor combined therapy based on nutritional support and corticosteroids. 9 We used oral prednisolone (30 mg/day) alone in the present case. One month after the initiation of prednisolone therapy the patient's symptoms markedly improved.…”
Section: Discussionmentioning
confidence: 99%