A Case of Enteritis Cystica Profunda in the Duodenum

Jeong, Ho Kyeong; Kim, Jin Kwan; Hwang, In Seog; Kim, Mi Young; Kim, Yeong Muk; Lee, Dong Yun; Lee, Joon Sang

Abstract

Enteritis cystica profunda (ECP) is a very rare condition of the small intestine. It is characterized by mucin-filled cystic space and partially lined by nonneoplastic epithelium predominant in the submucosa of the small bowel. It has the identical histopathologic features to those of colitis cystica profunda (CCP), which is a rare benign lesion of the distal colon and rectum of uncertain cause. ECP appears to occur much less than CCP and all reported ECP was associated with intestinal disease, such as Peutz-Jeghers syndrome, Crohn's disease, hamartomatous polyp, and multiple congenital anomalies. Among the reported ECP cases, only one case was located in duodenum and it was associated with hamartomatous polyps. Interestingly, we experienced a case of ECP in the duodenum without any intestinal disease. The diagnosis was made based on the endoscopic finding and histologic features of the resected specimen. We report this rare case of ECP located in the duodenum without associated small intestinal disease.