Case of the week: An unusual Constellation of Symptoms

A 63 year old man is referred to the internal medicine clinic for an unusual constellation of symptoms: 4 months of watery diarrhea, alopecia and nail loss.  He carries a hand written note from a gastroenterologist that states that he had innumerous polyps on a colonography done as part of his work up.  He has lost 15 kg in the past 3 months.

His nails look like this:

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What condition does he have?

He has a condition called Cronkhite-Canada Syndrome.  Although this is a very rare case, I have actually seen it once in my career so far!  It is an idiopathic condition that typically affects males more than females in the later decades of life.  It is associated with polyposis can present with abdominal pain, watery diarrhea and a protein losing enteropathy.  Additionally, there are characteristic nail changes (onychodystrophy) and macular skin discolouration on the hands which are demonstrated in the photo above. The hyperpigmented macular rash can also be seen on the feet, arms and face

It was discovered by Wilma Canada and Leonard Cronkhite and first published in the New England Journal of Medicine in 1955. The treatment is often symptomatic and involves hydration, nutrition and vitamin supplementation. The differential diagnosis includes Familial Adenomatous Polyposis and Peutz-Jeghers syndrome.

For more information, check out this great summary article on Cronkhite Canada Syndrome  from Rarediseases.org.

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