Nongranulomatous chronic idiopathic enterocolitis: a primary histologically defined disease

Nongranulomatous chronic idiopathic enterocolitis is characterized by sudden onset of severe watery diarrhea, malabsorption, exudative enteropathy, frequent appearance of shallow ulcerations, and variable degrees of villus atrophy. In the absence of infectious and pharmacologic causes, the presence of a predominantly acute inflammatory infiltrate limited to the lamina propria establishes the diagnosis. No underlying disease appears during prolonged follow-up. The etiology remains unknown. The disease is generally corticosteroid-responsive; low-dose maintenance therapy is frequently required. The long-term prognosis is guarded. Three of 11 patients died of opportunistic infections or resistance to therapy.