The Very Interesting Small Bowel Lesions of Cronkhite-Canada Syndrome

BACKGROUND: Cronkhite-Canada syndrome (CCS) is a non-hereditary disease characterized by the presence of gastrointestinal polyposis and skin symptoms such as hair loss, nail plate atrophy, and skin pigmentation. Approximately 50% of cases of CCS also have small bowel lesions. However, there have been few image reports of the small intestine lesions.

METHODS: We examined the findings of small intestine capsule endoscopy (CE) in 3 cases of CCS in our hospital.

RESULTS: Case 1 was of a 64-year-old male whose chief complaint was diarrhea, abnormal taste, and weight loss. He had diarrhea for 2 months and aware of finger pigmentation and nail atrophy. Esophagogastroduodenoscopy (EGD) revealed a collection of reddish polyps that expanded in the stomach and duodenum, and colonoscopy (CS) revealed glossy reddish bumps in the colon. CE showed diffuse reddish, edematous mucosa and villi enlargement in the entire small bowel. From these characteristic endoscopic and skin findings, he was diagnosed with CCS and was started on prednisolone (PSL) treatment. The treatment response was good, and there was no relapse. Case 2 was of a 65-year old female whose chief complaint was skin pigmentation. She had been aware of pigmentation on her back, palms, and upper arms, and loss of fingernails for the last 6 years with repeated disappearances and exacerbations. Furthermore, erosive gastritis and gastric hyperplastic polyps had been detected 4 years ago, and she was followed-up for these symptoms. Gradually, she became aware of nausea and loose stools, which prompted her to visit our hospital. EGD and CS revealed multiple reddish polyps predominantly in the gastric antrum, villous atrophy and reddish polyps in the terminal ileum, and scattered reddish polyps in the entire colon. Mucosal biopsies revealed glandular dilation, mucosal edema, and inflammatory cell infiltration. CE showed diffuse mulberry ridges and villi atrophy throughout the small bowel. She was diagnosed with CCS based on characteristic clinical and endoscopic findings, and was started on steroid treatment. There was no relapse. Case 3 was of a 75-year old male whose chief complaint was diarrhea and hair loss. EGD and CS revealed multiple polyps in the stomach, duodenum, and colon. CE showed reddish edematous mucosa and villi mixed with atrophy and swelling in the small bowel. Of note were that these findings were found in the jejunum. He was diagnosed with CCS and started on PSL and immunomodulator. Four years later, the characteristic findings disappeared in the CE.

CONCLUSION(S): All patients had lesions in the entire small bowel, but there they were not associated with clinical findings or steroid response. The mucosa of the small intestine were more likely to have various findings as the period from onset to diagnosis became longer.