Diagnosis and Discussion

Diagnosis

Enterocolic lymphocytic phlebitis.

Discussion

Enterocolic lymphocytic phlebitis (ELP) is characterized by localized phlebitis involving the bowel wall, preferentially the submucosa and mesentery, without involvement of arteries and no evidence of systemic vasculitis. ELP was first described in 1976 by Stevens et al. who described a case of necrotizing and granulomatous phlebitis involving the cecum and ascending colon. Subsequently, Saraga and Costa described a series of three cases in 1989 and coined the term ELP. ELP occurs in patients older than 50 years (median 63 years) and has been associated with comorbidities including cardiovascular disease, renal failure, and hypertension. The most common presentation is that of acute abdomen (sudden severe abdominal pain), but other symptoms may include diarrhea, nausea, vomiting, or rectal bleeding. In rare cases, ELP presents as a mass lesion and mimics malignancy. The etiology and pathogenesis of ELP are poorly understood. ELP has been associated with IgG4-related disease and microscopic colitis and/or enteritis, as well as the use of drugs, including rutoside and flutamide. ELP is treated, and in nearly all cases, cured by surgical resection of the involved segment of intestine.

Grossly, the colon shows non-specific changes such as infarction, bowel wall thickening, tumor-like masses, or ulceration. Microscopically, the hallmark of ELP is lymphocytic (T-cell) infiltration of the venous wall with eccentric or concentric perivascular lymphocytic cuffing, primarily involving the submucosa. A true necrotizing phlebitis with fibrinoid necrosis and neutrophilic infiltration of the vessel wall may also be seen, as well as granulomatous phlebitis with infiltration by giant cells and epithelioid histiocytes. Some vessels may contain early organizing venous thrombi, which likely cause intestinal ischemia and ulceration. Lastly, myointimal and endothelial hyperplasia may be present. Notably, since ELP is a submucosal process, the histologic findings will not be present on endoscopic mucosal biopsies, thus it typically is not diagnosed preoperatively. However, the endoscopic biopsy may show an ischemic pattern of injury.

The main histologic differential diagnosis to consider is involvement of the gastrointestinal tract by systemic vasculitis. This may be seen in patients with systemic lupus erythematosus, Henoch-Schönlein purpura, polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), granulomatosis with polyangiitis (formerly Wegener granulomatosis), and microscopic polyangiitis, among others. These patients often have a documented history of a systemic vasculitis and microscopically these vasculitides affect arteries and can involve veins, but not veins alone.

References

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Saraga E, Costa J. Idiopathic enterocolic lymphocytic phlebitis: a cause of intestinal ischemic necrosis. Am J Surg Pathol. 1989;13:303-308.

Stevens SM, Gue S, Finckh ES. Necrotizing and giant cell granulomatous phlebitis of cecum and ascending colon. Pathology. 1976;:259-264.

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