Idiopathic chronic ulcerative enteritis. Report of five cases and review of the literature

Abstract

Five patients with non-specific small intestinal ulceration and malabsorption are presented. Four of these patients had subtotal villous atrophy of jejunal mucosa but none showed a morphological improvement after gluten withdrawal from the diet. Intestinal ulceration caused the complications of melaena, intestinal obstruction and perforation which resulted in the death of three patients. A review of the English literature reveals twenty-seven similar cases for which the term idiopathic chronic ulcerative enteritis is recommended. The condition is of unknown aetiology, diagnosis only being firmly established by laparotomy and histological examination of resected bowel. The relationship of this syndrome to coeliac disease, intestinal lymphoma and Crohn's disease is discussed. Management is extremely difficult and the long-term prognosis poor. Gluten withdrawal should be tried in the presence of villous atrophy but the value of steroid therapy in unresponsive cases is unproven. Surgical excision of the worst affected segments of small bowel has so far proved to be the most effective course of action.