A Fatal Case of Calciphylaxis in a Patient with Systemic Lupus Erythematosus and Normal Renal Function.

To the Editor: Calciphylaxis is a rare condition that involves cutaneous microvascular calcification leading to thrombosis and occlusion. Initial lesions appear violaceous and progress to non-healing necrotic ulcerations1. One-year mortality is over 50%, with sepsis as the leading cause of death1. Calciphylaxis is well described in patients with endstage renal disease with abnormalities in the calcium-phosphate-parathyroid hormone (PTH) axis2. An increasing number of case reports describe calciphylaxis in the absence of renal impairment associated with connective tissue disease, primary hyperparathyroidism, malignancy, liver disease, and protein C or S deficiencies3. We describe a case of rapidly progressive nonuremic calciphylaxis in a patient with systemic lupus erythematosus (SLE) and highlight the importance of calciphylaxis in the differential diagnosis of cutaneous ulceration. Our patient was a 77-year-old white woman with a 20-year history of SLE with features of arthritis, positive antinuclear antibody (ANA), hypocomplementemia, pulmonary involvement, and Raynaud disease. Her immunosuppressive regimen included azathioprine and hydroxychloroquine. Her comorbidities included type II diabetes, recurrent venous thromboembolism treated with warfarin for over 10 years, coronary artery disease, pulmonary … Address correspondence to Dr. L.J. Albert, Division of Rheumatology, Department of Medicine, Toronto Western Hospital, 399 Bathurst St., 1E – 424, Toronto, Ontario M5T 2S8, Canada. E-mail: lori.albert{at}uhn.ca