Localized necrotizing angiitis of the ileum

Abstract

A 20-year-old woman was hospitalized repeatedly because of intermittent bouts of intestinal obstruction and the symptoms usually improved with conservative treatments. One year after the first admission the patient was hospitalized in emergency and a laparotomy revealed a circular stricture with a pinhole perforation in the ileum. Histological sections of the stricture showed the characteristic features of microscopic polyangiitis varying from active to resolving stages, which were localized in the ileum. Fibrinoid necrosis, fibroblastic and fibrous proliferation of the intima and fibrous replacement of the media with a variable pan- and perivascular inflammatory cell infiltrate were characteristic in the muscular arteries and arterioles. Vascular occlusion by pale eosinophilic, fibrillar-like materials resembling livedo racemosa of the skin, was noticed in the small arterioles and capillaries. Under no prophylaxis, the postoperative course was uneventful with no recurrence of the illness at an 18-month follow up. The pathological alterations were distributed focally, occasionally segmentally, and haphazardly, and required detailed examination by stepwise sections for the histological diagnosis.

Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades

“…49 In addition, there have been several case reports in the literature. [50][51][52][53][54] Burke et al, 43 in 1995, described a large series of 63 patients with localized GI vasculitis, but they included patients with SLE and RA, and many of their patients developed systemic vasculitis on follow-up, suggesting that theirs was a more heterogeneous group. Histologically proven granulomatous vasculitis with recurrences localized to the GI tract, as in our patient, is a very unusual entity.…”

“…The largest series of localized GI vasculitis consists of 18 patients . In addition, there have been several case reports in the literature . Burke et al ., in 1995, described a large series of 63 patients with localized GI vasculitis, but they included patients with SLE and RA, and many of their patients developed systemic vasculitis on follow‐up, suggesting that theirs was a more heterogeneous group.…”

Polyarteritis nodosa presenting as a febrile rash and severe fatal myocarditis

“…They may spread circumferentially to involve adjacent veins [4] . However, localized polyarteritis and vasculitis has been reported to occur in the muscles, gallbladder, pancreas, bowel and appendix [5][6][7] . The initial clinical syndrome was not typical of PAN.…”