Abstract

Rheumatoid vasculitis (RV) is a rare but severe complication of rheumatoid arthritis (RA). Despite its poor prognosis, the histopathologic findings of RV are not well defined. RV is typically described as a vasculitis of small and medium-sized vessels, with less attention given to its occlusive nature. Herein we present the histopathologic findings of both vasculitis and vasculopathy as key to diagnosis in a patient with RV. A 77-year-old man with RA presented with severe pain and discoloration of the fingertips, which quickly progressed to a mottled rash and ulcerations on the lower extremities. The condition continued to worsen despite multimodal therapy with steroids, antibiotics, vasodilators, and gentle wound care. Upon transfer to our hospital, he was noted to have livedo reticularis on all four extremities, well-demarcated ulcerations on the lower legs, and digital gangrene. Biopsy of a dusky lesion on the toe demonstrated both leukocytoclastic vasculitis and thrombotic vasculopathy with associated ischemic necrosis of the epidermis and eccrine glands. Special stains for microorganisms were negative. Rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) antibody were elevated at 281 IU/mL (0-15) and 672 U/mL (<20), respectively. A diagnosis of RV was made. Despite attempts to maximize therapy for RA, the patient succumbed to disease. Classically, the concomitant findings of vasculitis and vasculopathy would raise a differential diagnosis of mixed cryoglobulinemia, septic vasculitis/vasculopathy, and levamisole-induced vasculitis/vasculopathy. We propose the addition of RV to this group of vascular disorders in order to improve recognition and aid in timely diagnosis of this morbid disease.