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Nongranulomatous Chronic Idiopathic Enterocolitis

Pictures from the rectum and colon in a patient fulfilling the criterias for a Nongranulomatous Chronic Idiopathic Enterocolitis, with inflammation in the duodenum and in the colorectum and after having severe diarrhea for one year. The macroscopic finding is quite clearly a colitis, but it does not really look like Ulcerative Colitis, nor Crohn´s disease.

In 1996, Ruan et all reported 9 cases of a chronic inflammatory bowel disease, not fitting in into any previously known category, and probably representing a new, primary, not unfrequently fatal disease, where corticosteroid were of benefit. The typical features of this probably very rare disease are the following:
- severe chronic diarrhea with quite abrupt onset
- no infectious agent identified
- no underlying disease after extensive diagnostic investigations
- inflammation and sometimes superficial ulcerations in the duodenum, colon and rectum
- partial villous atrophy in duodenal biopsies - but no typical cellular abnormalities of the epithelial layer seen in celiac disease, and no response to gluten-free diet
- inflammatory changes in the colon mucosa, with crypt abscesses, but no granulomas (and no other features typical for Crohn´s disease, such as stenosis or fistulization.
- rapid and dramatic response to corticosteroid therapy, but prolonged therapy often needed.

Reference:

Ruan EA, Komorowski RA, Hogan WJ. Nongranulomatous Chronic Idiopathic Enterocolitis: Clinicopathologic Profile and Response to Corticosteroids. Gastroenterology 1996;111:629-637