Reactive angioendotheliomatosis of the intestine in Crohn’s disease: A second case report with 10-year follow up

Abstract

Reactive angioendotheliomatosis (RAE) is a rare cutaneous vascular disorder, usually associated with systemic diseases. RAE has previously been described only in the skin, with the single exception of one case involving the intestine in a patient with Crohn’s disease, associated with lower gastrointestinal bleeding. We report a second case of intestinal RAE, also associated with Crohn’s disease and bleeding, with 10-year follow up. A 48-year-old man with Crohn’s disease presented with fever and chills associated with an abscess-like lesion in the ileum. The patient underwent ileocecectomy. The specimen showed histologic features of Crohn’s disease. In addition, a florid vascular lesion was identified in the ileum, proximal to the area involved by Crohn’s enteritis. Histomorphology and immunophenotype of this vascular lesion was consistent with RAE. The lesion was intimately associated with mesenteric hematoma, suggestive of bleeding. No abscess was identified. Currently, 10years after surgery, the patient has persistent ileal Crohn’s enteritis, but otherwise remains healthy with no evidence of vascular disorder. Although rarely reported, intestinal RAE in Crohn’s disease may be under recognized. The co-existence of Crohn’s disease in our patient and prior case report suggests that immunologic factors underlie its pathogenesis. Pathologic recognition of the lesion and awareness of the existence of intestinal RAE in hemorrhagic forms of Crohn’s disease is warranted.