A Case of Idiopathic Chronic Ulcerative Enteritis Presenting with Septic Shock

Idiopathic chronic ulcerative enteritis (ICUE) is a rare disease with a mortality rate exceeding 75%, which manifests as an ulceration of the small bowel in the absence of a recognizable cause. It is diagnosed by biopsy. A 31-year-old man who had a 10-year history of recurrent episodes of abdominal pain was admitted with a 10-day occurrence of abdominal pain and fever. Upper endoscopy showed multiple active ulcers from duodenal second to distal portion. Colonoscopy revealed diffuse ulcerations at the terminal ileum. Colonic findings were normal. The patient was treated with intravenous antibiotics, systemic steroids, and total parenteral nutrition in the intensive care unit. Ten days after admission, the patient fell into shock due to massive hematochezia and underwent an emergency surgical resection, which revealed Meckel’s diverticulum. Bleeding was uncontrolled and the following day surgical resection and intraoperative endoscopy of the highly involved jejunum revealed severe ulcerative bleeding. Microscopic examination revealed ulcerations with pseudopolyps and granulation tissue and no evidence of vasculitis, suggestive of ICUE.