A 41-year-old African American woman with a 14-year history of systemic lupus erythematosus (SLE) presented with exquisitely painful stellate ulcers with surrounding livedo reticularis on both shins in April 2006. In addition to small-vessel involvement, her connective tissue disease was characterized by pulmonary fibrosis, pulmonary hypertension requiring continuous oxygen supplementation, Raynaud phenomenon, and chronic right popliteal deep venous thrombosis. Her medications included prednisone (5 mg/d), methotrexate (12.5 mg/wk), folic acid, hydroxychloroquine, pentoxyphilline, coumadin, budesonide inhaler, fluticasone inhaler, fexofenadine, erythropoietin, sildenafil, amlodipine, gabapentin, acetaminophen-codeine, tramadol hydrochloride, famciclovir, tolterodine, calcium supplement, vitamin E supplement, alendronate, and esomeprazole. The results of laboratory tests were remarkable for the following values: hemoglobin, 8.8 g/dL (to convert to grams per liter, multiply by 10.0); hematocrit, 27.1% (reference range, 34%-45%); antinuclear antibody, 1:1280 speckled; double-stranded DNA, 52.1 IU/mL (reference value, <5 IU/mL); nRNP/Sm IgG autoantibodies, 118.0 U (<5.0 U); Sm IgG autoantibodies, 114.0 U (reference value, <5.0 U); SSA IgG autoantibodies, 158.0 U (reference value, <5.0 U); SSB IgG autoantibodies, 167.0 U (reference value, <5.0 U); C3 complement, 49 mg/dL (reference range, 82-235 mg/dL), anticardiolipin antibody IgG, 50 IgG phospholipid units (reference value, <15 IgG phospholipid units); and iron, 13 μg/dL (to convert to micromoles per liter, multiply by 0.179) (reference range, 40-170 μg/dL). The findings of urinalysis were normal, as were renal and liver functions. Magnetic resonance imaging of the bilateral lower extremities showed no evidence of osteomyelitis. On examination, the distal portion of the patient's extremities were cyanotic and cold, with sclerodactyly, finger pad atrophy, and bone resorption. The shin ulcers were 10.0 × 12.5 cm bilaterally.