About gastrointestinal polyposis and ectodermal changes

What is gastrointestinal polyposis and ectodermal changes?

Cronkhite-Canada syndrome (CCS) is a very rare disease with symptoms that include loss of taste, intestinal polyps, hair loss, and nail growth problems. It is difficult to treat because of malabsorption that accompanies the polyps. CCS occurs primarily in older people (the average age is 59) and it is not believed to have a genetic component. There have been fewer than 400 cases reported in the past 50 years, primarily in Japan but also in the U.S. and other countries.

What are the symptoms for gastrointestinal polyposis and ectodermal changes?

The symptoms of Cronkhite-Canada syndrome occur because of multiple polyps occurring in the stomach, small intestine, colon and, less frequently, the esophagus. These include chronic or recurring watery Diarrhea, cramps, and abdominal discomfort. These people may also have abnormally low levels of protein in the blood (protein-losing enteropathy), causing a feeling of general ill health (cachexia), malnutrition, Nausea and Vomiting.

The earliest symptoms reported are changes in taste and loss of smell. Patients can even experience a profound loss of appetite, sometimes to the point of malnutrition, weight-loss and/or excess fluid accumulation in the arms and legs (peripheral edema). An imbalance of certain essential minerals (electrolytes) may occur because of chronic Diarrhea. Some people with CCS may also have large skin bruises (ecchymotic plaques) and/or impaired lung function. Other symptoms may include loss of hair (alopecia), large areas of dark spots on the skin (hyperpigmentation) and degenerative changes and, eventually, loss of the fingernails (onychodystrophy).

Patients with CCS can also have coexisting autoimmune disorders, where the body develops antibodies against an organ, thereby attacking itself, e.g. hypothyroidism, rheumatoid arthritis, scleroderma, systemic lupus erythematous, etc.

What are the causes for gastrointestinal polyposis and ectodermal changes?

The exact cause of Cronkhite-Canada syndrome is unknown. It seems to occur for no known reason (sporadically) and is not thought to be hereditary.

What are the treatments for gastrointestinal polyposis and ectodermal changes?

Treatment is based on controlling symptoms and providing support. The primary goal of treatment is to correct fluid, electrolyte and protein loss by nutritional supplementation or a nutritionally balanced liquid diet. Corticosteroids (i.e., prednisone) may be given occasionally to help reduce intestinal inflammation. Bacterial overgrowth in the intestines, which can cause malabsorption, may be treated with antibiotics. In rare cases, symptoms have resolved for no apparent reason (spontaneous remission). Surgical removal of polyps may help to relieve some of the symptoms of Cronkhite-Canada syndrome. However, they may recur or be too numerous to remove individually. If necessary, severely affected portions of the colon may be removed. Case reports have suggested the use of immunosuppressive treatment, including aziathioprin and ciclosporin, if other treatments are not effective.

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