cronkhite - canada syndrome ( ccs ) was first described in 1955 by cronkhite and canada . the main symptoms of ccs are diarrhea , weight loss , abdominal pain , and other gastrointestinal complications , such as protein - losing enteropathy and malnutrition with diffuse polyposis in the digestive tract . ccs is a rare non - genetic disease of unknown cause characterized by alopecia , atrophic fingernails , and skin hyperpigmentation . in the past , ccs was regarded clinically as a malignancy because of its poor prognosis , but recent advances such as high - calorie infusion and steroid therapy have achieved long - term survival in some cases . ccs was considered a benign condition , however , there are several case reports of ccs associated with gastrointestinal carcinoma [ 2 , 3 , 4 ] and , since the etiology of this syndrome remains unknown , many cases are refractory to treatment and there are still fatalities . therefore , new therapies are urgently needed . our patient initially presented with diarrhea , and sessile polyposis was revealed in the colon , intestine and stomach by colonoscopy and upper gastrointestinal endoscopy . after successful eradication of h. pylori , she underwent ileostomy closure and complete polyposis resolution has since been observed . we describe this rare case with complete regression of ccs lesions and consider novel treatments for ccs . a 52-year - old woman , previously in good health without symptoms , developed diarrhea with lower abdominal pain during bowel movements and hematochezia , which continued for more than 2 weeks , after ingesting oysters and goat meat . at our hospital , colonoscopy demonstrated diffuse edema and a markedly erythematous elevated lesion from the end of the ileum to the rectum ( fig . after hospitalization , infectious enteritis was suspected , but stool culture showed no obvious pathogens and there were no parasites in stool specimens . upper gastrointestinal endoscopy showed salmon roe - like multiple elevated lesions with marked erythema involving almost the entire stomach ( fig . 2b ) , similar to that in the colon , as well as similar mild polypoid lesions in the duodenum . initial pathological findings showed non - specific chronic inflammatory cell infiltration , edema and thickening of the colonic mucosa and crypts with cystic dilatation in the intestine ( fig . however , neither foveolar gland hyperplasia nor interstitial inflammatory cell infiltration was found in the stomach . her symptoms persisted after starting 5-asa and she also showed alopecia and glossitis 1 month later . at the second colonoscopy , an elevated tumor 30  35 mm was observed in the sigmoid colon ( fig . 1b ) . since the biopsy specimen was consistent with group 5 , a well - differentiated adenocarcinoma , high anterior resection of the sigmoid colon and ileostomy were performed . even when these treatments were stopped , the diarrhea , alopecia and glossitis continued to gradually improve . detailed examination of the surrounding mucosa revealed cystic dilatation of glands and edema , infiltration of eosinophils with plasma cells and lymphocytes , and spread of lymphangitis . ccs associated with sigmoid colon cancer ( 01 , pm n0 m0 ) with tubular adenoma was thus diagnosed ( fig . colonoscopic observation via the ileostomy , after surgery , revealed amelioration of the edema and erythema in the ileum , ileocecal region and ascending colon . on colonoscopy 6 months later , the findings in the ileum had almost disappeared and erythematous polypoid lesions and edematous mucosa showed regression in the colon . although spread of cystic crypts was observed in some portions of the intestine , colonic histology , i.e. mucosal thickening , showed improvement 1 year later . gastric foveolar hyperplasia and edema disappeared , and mucosal thickness was normal in the stomach . on the other hand , on colonoscopy 2 years later , while polyps were no longer visible , erythema and petechiae persisted , reflecting diversion colitis ( fig . the thickness of the colonic mucosa was histologically normal with no edema , but extended cystic crypts remained in portions of the intestine . upper gastrointestinal endoscopy confirmed the absence of gastric polyps or other abnormal findings in the duodenum ( fig . 2c ) . histopathological features of the stomach indicated normal thickness without mucosal edema , and chronic gastritis with positive h. pylori histology and serology . after 4 months , the endoscopic and histological findings of gastritis had also improved and active inflammation had disappeared ( fig . surgical closure of an ileal fistula was carried out and , after 4 months , upper gastrointestinal endoscopy and colonoscopy showed that not only the ccs lesions but also the diversion colitis findings had completely disappeared . as the patient remained asymptomatic during observation , she has since been followed at our outpatient clinic without medication . after 2 further years , colonoscopy and upper gastrointestinal endoscopy showed normal findings ( fig . the number of ccs cases in asia , including japan , is reportedly high . although ccs has been recognized as a benign non - genetic disease , it manifests clinically as protein - losing gastroenteropathy , and relapse or exacerbation is common despite temporary improvements with medical therapy , such as steroids . ccs was formerly considered to have a poor prognosis and a high mortality rate due to the complication of carcinoma , and many patients present with malignancy . although the characteristics of ccs polyps are considered to be non - neoplastic in nature , a relatively high frequency of gastrointestinal carcinoma in ccs patients has been reported [ 2 , 3 , 4 , 5 ] . the course in our case was also complicated by early - onset carcinoma necessitating partial resection of the sigmoid colon and ileostomy . subsequently , symptoms and endoscopic and histological findings showed amelioration , and the patient was followed without further intervention . she underwent h. pylori eradication 2 years after surgery and the ileostomy was closed the following year , after h. pylori eradication . three months after this closure , ccs lesions of the colon and stomach were no longer visible macroscopically or histologically . , there are no prior reports of ccs cases such as our present patient undergoing surgical treatment and eradication of h. pylori . steroids have commonly been administered for ccs and cases achieving temporary improvement or even long - term remission have been reported . however , there were numerous recurrences and effectiveness , as compared to other treatments , has not been established . among reports supporting surgical treatment of ccs therapeutic effects would presumably have been obtained if the site in the digestive tract , harboring polyposis and causing protein leakage , had been resected along with the carcinoma . reported in their literature review that symptomatic remissions were observed in 2 of 6 cases who underwent subtotal gastrectomy alone . in addition , symptomatic remissions were attributed to the operations alone in 3 of 7 patients receiving large bowel resection , among 55 ccs cases . in our case , only partial resection of the sigmoid colon was performed for tumor removal from the sigmoid colon and polyposis remained at other sites . therefore , it is possible that changes in bacterial dynamics within the intestinal flora are related to the improvement achieved by ileostomy in our patient . furthermore , clear evidence implicating h. pylori infection in the pathogenesis of ccs is lacking . recently reported that lesions such as diffuse polyposis showed progressive remission after h. pylori eradication using antibiotics in patients with ccs . h. pylori usually inhabits the gastric mucosa , but this microorganism has reportedly been cultured from stool specimens of h. pylori carriers . h. pylori might injure the intestinal mucosa when passing through the intestinal tract , and/or activation of lymphocytes in the gastric mucosa by h. pylori may induce an immune response against this microorganism in the intestinal mucosa . as with maltoma of the stomach , based on these reports and our evidence , we can not rule out the possibility that h. pylori infection is involved in the development or exacerbation of ccs polyps , but the underlying mechanism remains uncertain . accumulation of such cases and further research are necessary to explain the pathogenesis of ccs and to devise novel treatments for this disease .