Cutaneous vasculitis in lupus treated with IV immunoglobulin

Abstract

A 44-year-old man presented to the emergency department with a 3-week history of painful ulcers in the sacral region with a new skin rash. He had received an early diagnosis of systemic lupus erythematosus (SLE) at the age of 7 years and was using only prednisone 15 mg daily (he had stopped using hydroxychloroquine by himself 3 months earlier). On physical examination, multiple necrotic ulcers were noted with major livedo racemosa intermingled (Fig. 1a). The systemic examination was unremarkable. A differential diagnosis workup between vasculitis and antiphospholipid syndrome was performed, and a skin biopsy was promptly collected. Histopathologic examination revealed diffuse neutrophilic infiltrate around small vessels (Fig. 1c). Direct immunofluorescence showed moderate homogeneous deposits of both immunoglobulin M (Fig. 1d) and C3 (Fig. 1e) along the basement membrane zone. Laboratory tests revealed hemoglobin 11.8 g/dL; an elevated erythrocyte sedimentation rate of 47 mm, C-reactive protein 10 mg/dL, creatinine 1 mg/dL, urea 30 mg/dL, cryoglobulin negative, low C3 of 39 mg/dL and C4 of 8 mg/dL, ANCA and serology tests for HIV, hepatitis B and C, and VDRL were negative, ANA 1/640 nuclear homogeneous, anti-Smith > 180 IU/mL, anti-SSA > 240 IU/ mL, anti-SSB > 320 IU/mL, anti-dsDNA > 379 IU/mL, negative anti-phospholipid antibodies (anti-cardiolipin, anti-beta 2 glycoprotein I, and lupus anticoagulant), urinalysis without significant proteinuria; and no dysmorphic erythrocytes. We opted to start prednisone 1 mg/kg daily with hydroxychloroquine 400 mg/day, and an improvement in the livedo was observed. However, the patient’s ulcers became infected during the hospital stay (Fig. 1b), demonstrated by a positive culture for Pseudomonas aeruginosa, and they were treated with culture-guided ciprofloxacin and surgical debridement. Due to clinical instability from sepsis, a second immunosuppressive agent was reconsidered, and we chose to start immunoglobulin 1 g/kg for two consecutive days. Unfortunately, soon after, the patient died from septic shock.