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SLE: systemic lupus erythematosus INTRODUCTION Calciphylaxis, or calcific uremic arteriolopathy, is a rare disorder characterized by calcium deposition in both dermal and subcutaneous adipose tissue as well as in small vessels. It leads to thrombosis, ischemia, and cutaneous necrosis. Calciphylaxis is typically seen in the setting of end-stage renal disease, but it has been increasingly reported in patients with preserved renal function and patients with systemic lupus erythematosus (SLE). We report an unusual, highly aggressive, and rapidly progressive case of fatal nonuremic calciphylaxis with atypical clinical and histopathologic features in a patient with SLE.

A unique clinical and histologic presentation of catastrophic systemic calciphylaxis in a nonuremic patient with systemic lupus erythematosus