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June 2009

Calciphylaxis Associated With Chronic Inflammatory Conditions, Immunosuppression Therapy, and Normal Renal Function: A Report of 2 Cases

Arch Dermatol. 2009;145(6):723-725. doi:10.1001/archdermatol.2009.103

Calciphylaxis is a syndrome of vascular calcification and skin necrosis commonly associated with chronic renal failure. We present 2 cases of calciphylaxis in patients with chronic inflammatory conditions and immunosuppression but normal renal function.

Case 1. A 54-year-old white man presented with a 4-month history of a worsening ulcer on the right lower extremity (Figure 1). A punch biopsy specimen revealed fungal elements in the epidermis and superficial dermis and calcium deposits in the media of blood vessel walls. Wound cultures grew Aspergillus and Pseudomonas, and the patient was admitted to the hospital for antibiotic and antifungal treatment. Medical history revealed that the patient had a 3-year history of chronic inflammatory demyelinating polyneuropathy associated with hepatitis C and IgA monoclonal gammopathy. The patient was undergoing drug treatment with high-dose prednisone, mycophenolate mofetil, and monthly intravenous immunoglobulin infusions for this condition. The patient also had protein S deficiency and had been undergoing warfarin therapy for the past 3 years.

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