Case Report
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World J Gastroenterol. Jun 21, 2014; 20(23): 7518-7522
Published online Jun 21, 2014. doi: 10.3748/wjg.v20.i23.7518
Cronkhite-Canada syndrome: Report of six cases and review of literature
Xiao-Heng Wen, Lan Wang, Yu-Xuan Wang, Jia-Ming Qian
Xiao-Heng Wen, Lan Wang, Jia-Ming Qian, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China
Yu-Xuan Wang, Department of Behavioral Sciences and Health Education, Rollins School of Public Health, Emory University, Atlanta, GA 30322, United States
Author contributions: Wen XH and Qian JM designed the study; Wen XH and Wang L collected the patient’s clinical data, analyzed the data and wrote the paper; Wang YX and Qian JM revised the paper.
Correspondence to: Jia-Ming Qian, Director, Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No. 1, Shuai Fu Yuan, Beijing 100730, China. qianjiaming1957@126.com
Telephone: +86-10-65295019 Fax: +86-10-65295019s
Received: January 14, 2014
Revised: February 22, 2014
Accepted: April 8, 2014
Published online: June 21, 2014
Processing time: 157 Days and 22 Hours
Abstract

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.

Keywords: Cronkhite-Canada syndrome; Clinical feature; Etiology; Therapeutics

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.