Cronkhite–Canada syndrome

Author: Brian Wu, MD candidate, Keck School of Medicine, Los Angeles, USA. DermNet New Zealand Editor in Chief: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. December 2016.

What is Cronkhite–Canada syndrome?

Cronkhite–Canada syndrome (CCS) is a rare, sporadic, non-inherited disorder characterised by gastrointestinal and dermatological symptoms.

It has a number of other names, including Canada–Cronkhite syndrome, Cronkhite–Canada polyposis, allergic granulomatous angiitis of Cronkhite–Canada, gastrointestinal multiple polyposis syndrome, and gastrointestinal polyposis and ectodermal changes. 

CCS was first described by Leonard Cronkhite and Wilma Canada in 1955.

Who gets Cronkhite–Canada syndrome?

CCS is extremely rare:

What causes Cronkhite–Canada syndrome?

The exact cause of CCS is unknown: some researchers have attributed it to immune dysfunction, although in some studies, it has been linked to arsenic poisoning.

What are the clinical features of Cronkhite–Canada syndrome?

Gastrointestinal problems seen in CCS include:

The ectodermal changes seen in CCS are thought to be due to malabsorption and malnutrition; these include:

<p">Other disorders found in CCS include:

How is Cronkhite–Canada syndrome diagnosed?

The diagnosis of CCS can be reached after a variety of tests are performed. These tests include:

How is Cronkhite–Canada syndrome treated?

Because there is no cure for CCS, treatment is symptomatic. Treatment focusses on hydration, restoration of nutrients and electrolytes, and regularisation of bowel movements. Pain management may also be required. Regular colonoscopy to remove polyps and identify colon cancer should be undertaken.

Treatments of bowel symptoms may include:

What is the outlook for Cronkhite–Canada syndrome?

The outlook for CCS is poor, with mortality rates in the first 5 years after diagnosis of around 50%, even with treatment. This is usually due to colon cancer or portal vein thrombosis.

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