CRONKHITE-CANADA SYNDROME
AND OTHER RARE DIARRHEAL
DISORDERS
Cronkhite-Canada Syndrome
Cronkhite-Canada
syndrome is a rare, nonfamilial gastrointestinal polyposis syndrome that
commonly presents with diarrhea. It is characterized by hyperpig-mentation,
hair loss, and dystrophic changes in the fingernails. Individuals of European
and Asian descent are most frequently affected, but the syndrome has been
reported in all ethnic groups. The cause is not known, but the frequent
association with hypothyroidism, systemic lupus erythematosus, rheumatoid
arthritis, and scleroderma suggests an autoimmune origin. The syndrome is
characterized by development of innumerable polyps throughout the
gastrointestinal tract except in the esophagus. The polyps are hamartomas;
there is an increased risk of colorectal cancer (25%), however. Patients
commonly present with diarrhea and weight loss accompanied by typical
dermatologic manifestations that include hyperpigmentation and onychodystrophy.
Management is largely supportive, with nutritional support, accompanied by
antisecretory and antiinflammatory treatment. Immunosuppressive therapy with
glucocorticosteroids and azathioprine has also been reported to ameliorate
symptoms, but the duration of treatment is not known.