Cronkhite-Canada syndrome

Cronkhite-Canada syndrome is an extremely rare disorder in which numerous polyps develop in the stomach, small intestine and colon, and less often in the esophagus, together with changes in the skin, hair and nails. The first symptoms reported are usually a change in taste and a loss of smell, followed by chronic or recurring watery diarrhea, cramps, abdominal discomfort and a profound loss of appetite. Protein loss through the bowel and poor absorption of nutrients can cause malnutrition, weight loss and swelling of the arms and legs. The skin signs are loss of scalp hair, large areas of darker skin and degeneration and eventual loss of the fingernails. The condition usually appears in middle or old age, with an average onset near 59 years, and affects men somewhat more often than women, at roughly three to two. More than 500 cases have been reported worldwide, mostly in Japan. The exact cause is unknown and the disease is considered acquired rather than hereditary. Treatment aims to correct fluid, electrolyte and protein loss and can include corticosteroids, antibiotics for bacterial overgrowth, and surgical removal of polyps.