Refractory cutaneous polyarteritis nodosa with threat of loss of feet: successful treatment with rituximab

Abstract

Cutaneous Polyarteritis Nodosa (CPAN) is a rare, single organ vasculitis of unknown etiology which presents with livedo reticularis and tender nodules involving the lower extremity in 97% of cases. Diagnosis is established by skin biopsy demonstrating segmental leukocytoclastic vasculitis and fibrinoid necrosis with workup excluding any systemic involvement ( Figure 1). The presence of IgM antiphosphatidylserine-prothrombin complexes has led to the hypothesis that CPAN is a localized arthrus reaction. CPAN is characterized as a benign, chronic, relapsing vasculitis to be treated conservatively. As a single organ vasculitis, CPAN can affect arteries of any size. Due to CPAN's predilection for the lower extremities, involvement of the limb at or below the ankle's major arteries may result in loss of digits and potentially feet. The case presented involves CPAN in which the main arteries to the feet are occluded. The patient had been refractory to therapy with findings of early digital necrosis. Following adequate skin biopsy to establish a diagnosis, we initiated targeted therapy with rituximab, azathioprine, and high dose steroids with reversal of the ischemic process. It is the authors' opinion that CPAN should be treated more aggressively from initial diagnosis to prevent morbidity and potentially abort a chronic relapsing course.