Cronkhite- Canada Syndrome (CCS): Be Aware of Rapid Hair Loss Following Diarrhea and Loss of Taste

Cronkhite- Canada Syndrome (CCS) : Another Mimicker of Many Diseases

Cronkhite- Canada Syndrome (CCS) is a condition that occurs in adults. There are no specific diagnostic tests so recognizing all the features is key to getting the diagnosis. Not surprisingly, many go undiagnosed. CCS was first reported in 1955 by Dr Cronkhite and Dr Canada.

The cause is not clear but it may be autoimmune (many patients have elevated IgG4 and autoimmune markers like positive ANA). The mean age of affected patients is around 60 years although it does range from the 30s to 80s. Males are slightly more affected than females. It happens to people worldwide but many cases in the medical journals are from Japan.

Patients develop a range of symptoms early on in the disease course including large volume diarrhea and abdominal pain, loss of taste and smell, loss of appetite, weight loss and weakness. Diarrhea and lost of taste are often the first symptoms.

This is followed by the “dermatologic triad” of nail changes (even loss of nails), hair loss and pigmentation changes on palms, soles, chest, and face. Hair loss can be rapid and mimic alopecia totalis. In some patients, these changes happen before the diarrhea but usually happens weeks to months after the diarrhea and abdominal pain started.

Many feel unwell with nausea and vomiting and do not wish to eat. Fluid retention is frequent.

Some patients have neurological issues like numbness and tingling.

Polyps are found in the colon, stomach and small bowel. The pathology of the polyps is that of non cancerous hamartomas. Colon cancer develops in about 10 % of patients.
Many patients develop autoimmune disorders.

Treatment is with steroids, immunosuppressants (like azathioprine) and immune modulators (TNF inhibitors) and good attention to nutrition and balancing electrolytes. Mortality can still be quite high and many patients die of malignancy, portal vein thrombosis, infection or kidney disease.

Summary

Cronkhite- Canada Syndrome (CCS) is often misdiagnosed or severely delayed in its diagnosis. We need to be aware fo the patient who goes from healthy to suddenly feeling unwell, not wanting to eat and having chronic large volume diarrhea followed by the “dermatologic” triad of hair loss, nail changes and pigmentation changes. This may be a sign of CCS.