Cronkhite–Canada disease

Cronkhite–Canada disease is a rare syndrome characterised by multiple polyps of the digestive tract. It is sporadic (i.e. it does not seem to be a hereditary disease), and it is currently considered acquired and idiopathic (i.e. cause remains unknown).

About two-thirds of patients are of Japanese descent and the male to female ratio is 2:1.

It was characterized in 1955.

Presentation

Polyps are most frequent in the stomach and large intestine, are also found in the small intestine, and are least frequent in the oesophagus. A biopsy will reveal them to be hamartomas; the possibility that they progress to cancer is generally considered to be low, although it has been reported multiple times in the past. Chronic diarrhoea and protein-losing enteropathy are often observed. Possible collateral features include variable anomalies of ectodermal tissues, such as alopecia, atrophy of the nails, or skip pigmentation of the skin.

Cronkhite-Canada syndrome is a rare disease affecting mainly patients between 50-60 years of age, characterised by skin changes and gastrointestinal distrubances. Patients present with epidermal changes such as hair loss, nail loss, and hyperpigmentation. There can also be varying degrees of malabsorption, malnutrition, hematochezia, and in some cases, colorectal cancer.

Cause

The cause of the disease is unknown. It was originally thought that the epidermal changes were secondary to profound malnutrition as a result of protein-losing enteropathy. Recent findings have called this hypothesis into question; specifically, the hair and nail changes may not improve with improved nutrition.

Treatment

Treatments proposed include cromolyn sodium and prednisone.