Published online Jun 21, 2014. doi: 10.3748/wjg.v20.i23.7518
Revised: February 22, 2014
Accepted: April 8, 2014
Published online: June 21, 2014
Cronkhite-Canada syndrome (CCS) is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin. The aim of this study was to investigate the clinical features and potential therapies for CCS. Six patients with CCS admitted from December 1992 to July 2008 to Peking Union Medical College Hospital were evaluated. All patients had clinical manifestation of nonhereditary gastrointestinal polyposis with diarrhea, skin hyperpigmentation, alopecia, and nail dystrophy. Fecal occult blood was positive in all six cases. Serum hemoglobin, potassium, calcium and protein were below the normal range in two cases. Anti-Saccharomyces cerevisiae and antinuclear antibodies were present in three cases. Multiple polyps were found in all patients by gastroscopy and colonoscopy, with only one in the esophagus. Histologically, there were hyperplastic polyps in five cases, tubular adenoma in three, and juvenile polyp in one with chronic inflammation and mucosal edema. Comprehensive treatment led by corticosteroids can result in partial remission of clinical symptoms, and long-term follow-up is necessary.
Core tip: Cronkhite-Canada syndrome (CCS) is a rare noncongenital gastrointestinal polyposis syndrome, characterized by skin hyperpigmentation, hair loss and nail atrophy, associated with high morbidity. This case report summarizes the characteristics of six CCS patients, and reviews the literature. Comprehensive treatment led by corticosteroids can improve prognosis, and long-term follow-up is necessary.