CCS is a rare non-hereditary disorder characterized by 1) gastrointestinal polyposis and 2) characteristic ectodermal changes (hair, skin, nail, and tongue). Since the first report of two patients by Cronkhite and Canada,1 more than 400 cases have been reported worldwide, and the majority of them were in Japan.2, 3 Until now, 15 cases of CCS have been reported in Korea.4, 5 According to previous reports, the mean age of patients at the time of diagnosis was between late 50s and early 60s, with greater occurrence in males (male to female ratio was 1.3–1.84:1).2, 6 Pathophysiology of CCS is still unclear, but some authors have suggested mental stress and physical fatigue as possible precipitating factors. Common complications are malnutrition, gastrointestinal bleeding, and infection. The overall prognosis is poor, and the mortality rate is reported to be as high as 55%, if not properly treated.6 Recently, the autoimmune process is considered as the main pathophysiologic factor of CCS due to the good response in steroid treatment and IgG4-positive cells in tissue biopsy.7

Common presenting symptoms of CCS are diarrhea, weight loss, abdominal pain, and anorexia. Characteristic ectodermal changes, such as nail changes, hair loss, and skin hyperpigmentation are present in more than 70% of cases.6 Polyps can involve the entire gastrointestinal tract, from the esophagus to the rectum; however, the esophagus is usually rarely involved (2–12%).2, 6 Histologic findings of polyps are usually nonspecific, showing hyperplastic polyp, hamartomatous or juvenile polyp, inflammatory polyp, and tubular adenoma.4 At the initial presentation or during the follow-up period, gastrointestinal malignancies, such as colon or gastric cancer, are reported (15–25%).5 However, whether polyps in CCS possess malignant potential has been controversial due to the difficulties distinguishing between premalignant adenomas and multiple inflammatory pseudopolyps.2

Given its rarity, standard treatment of CCS has not yet been established. However, steroid administration accompanied by nutritional support is known as the mainstay of treatment. Regression of polyposis and achievement of sustained endoscopic remission are the purpose for treating CCS. Watanabe et al. suggested that good early response (regression of polyposis within 6–12 months after the start of treatment or initial diagnosis) and good long-term response (maintenance of endoscopic remission over a 3-year period after initial treatment) are related with favorable prognosis.2 Administration of histamine H2 receptor antagonist, proton pump inhibitor, 5-aminosalicylic acid, antibiotics, and Helicobacter pylori eradication also showed good results in previous reports.4 During the follow-up period, CCS can recur even in patients with successful discontinuation of steroid and clinical remission; thus, minimal annual endoscopic surveillance is usually recommended and maintenance treatment using a low-dose steroid, immunomodulator, and anti-tumor necrosis factor should also be considered.

In conclusion, CCS may be associated with significant complications, such as gastrointestinal bleeding, infection, and malignancy. However, early detection and proper steroid administration can achieve rapid clinical improvement and regression of polyposis, which can bring better overall outcome of CCS, as we have seen in this case. During long-term follow-up, recurrence of polyposis and CCS-related cancer can develop; thus, regular endoscopic surveillance is recommended.