A Constellation of Contemporaneous Comorbid Etiologies of Vasculopathy in a Patient With Rheumatoid Arthritis

Introduction. A 61-year-old man was hospitalized for worsening cutaneous ulcerations on his head, extremities, and trunk.

History. The patient had a 40-year medical history significant for rheumatoid arthritis (RA), Sjogren syndrome, and monoclonal gammopathy of undetermined significance (MGUS). At the time of admission, his home medication regimen included leflunomide 20 mg and prednisone 15 mg, both daily. A physical examination demonstrated broad geometric ulcerations with overlying fibrinous necrosis on the head, extremities, and trunk, along with extensive fibrosis and subcutaneous calcific changes of the trunk and shoulders (Figures 1-2). No significant abnormal pulses were noted.

Figure 1. The patient’s initial clinical presentation of broad, geometric, ulcerated plaques with fibrinoid necrosis and perilesional erythema on the scalp.

Figure 2. The patient’s initial clinical presentation of woody indurated fibrosis with calcific changes and retiform purpura of trunk and focal ulcerations with necrosis.

Diagnostic testing. Laboratory values, cultures, and skin biopsies were collected. The patient was cryoglobulin-positive (type not specified) and antinuclear antibody–positive (with negative titer). Serum protein electrophoresis demonstrated elevated free kappa and lambda light chains. Urine protein electrophoresis showed elevated gamma globulin M spike. The patient had negative immunofixation. Hypercoagulability, autoimmune, and nutritional deficiency work-up was otherwise negative. Calcium-phosphorus product was within normal limits. Scalp tissue culture grew pseudomonas aeruginosa and prevotella bivia.

Scalp and mandible biopsies demonstrated epidermal ulceration with dermal ischemia and brisk acute and chronic inflammation. Due to underlying RA and MGUS and detectable cryoglobulinemia, the patient's condition was diagnosed as thrombotic vasculopathy. Truncal biopsy demonstrated pannicular vascular calcification with thrombi and extravascular pannicular calcium deposits, diagnostic for calciphylaxis (Figure 3).