Idiopathic chronic ulcerative enteritis. Report of five cases and review of the literature
Mills, P.R.; Brown, I.L.; Watkinson, G.
Quarterly Journal of Medicine 49(194): 133-149
1980
ISSN/ISBN: 0033-5622 PMID: 7433632 Document Number: 167221
Five patients with nonspecific small intestinal ulceration and malabsorption are described; 4 of them had subtotal villous atrophy of jejunal mucosa, but none showed morphological improvement after gluten was withdrawn from the diet. Intestinal ulceration caused the complications of melaena, intestinal obstruction and perforation which resulted in the death of 3 patients. A review of the English-language literature shows 27 similar cases for which the term idiopathic chronic ulcerative enteritis is recommended. The condition is of unknown aetiology, diagnosis being firmly established only by laparotomy and histological examination of resected bowel. The relation of this syndrome to coeliac disease, intestinal lymphoma and Crohn's disease is discussed. Management is extremely difficult and the long-term prognosis is poor. Gluten withdrawal should be tried in the presence of villous atrophy but the value of steroid treatment in unresponsive cases is not proven. Surgical excision of the worst affected segments of small bowel is so far the most effective course of action.