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 with our own picture documentation of this rare condition.

Highlights

  • HistoryCronkhite-Canada syndrome (CCS) is a rare disease; about 450 cases have been described in the literature so far

  • In 1972, Johnson et al [3] published that the polyps in the stomach and large intestine are hamartomas and confirmed the description of Jarnum and Jensen

  • Goto divided the disease into five groups according to the leading symptom in 1995 [4]; type 1: diarrhoea is dominant, type 2: dysgeusia, type 3: abnormal sensation in the mouth with thirst, type 4: abdominal symptoms other than diarrhoea, and type 5: alopecia as a main symptom

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Summary

History

Cronkhite-Canada syndrome (CCS) is a rare disease; about 450 cases have been described in the literature so far. The disease was first described in 1955 by the American internist Leonard Wolsey Cronkhite and the American radiologist Wilma Jeanne Canada in the New England Journal of Medicine. They published two cases of an unusual fatal syndrome of diarrhoea, nausea, vomiting, and abdominal pain in a 42-year-old female and a 75-year-old female. Jarnum and Jensen [2] established the term CronkhiteCanada syndrome in their publication in 1966 They published a case report with two new observations in CCS patients: protein-losing enteropathy with electrolyte disturbances (hypocalcaemia, hypomagnesaemia, and hypokalaemia) and presence of nonadenomatous cystic polyps [2]. The mean age of onset is estimated to be in the fifth to sixth decade with a slight male predominance in the ratio 3 : 2 [6]

Etiology and Clinical Features
Histopathology
Complications and Prognosis
Treatment
Differential Diagnosis
Findings
Conclusion
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