Systemic calciphylaxis in a patient with end-stage renal disease and a normal parathyroid hormone level taking erythropoietin intramuscularly.

ABSTRACT

OBJECTIVE: To describe a patient with systemic calciphylaxis. METHODS: We present a case of a 26-year-old woman with end-stage renal disease taking intramuscular injections of erythropoietin in whom systemic calciphylaxis subsequently developed. Both clinical and laboratory findings are reviewed. RESULTS: In a 26-year-old woman with end-stage renal disease who was taking erythropoietin intramuscularly, multiple, superficial ulcerative lesions developed on both lower extremities. Despite aggressive treatment, new lesions subsequently developed. Laboratory values showed an increased calcium-phosphate product and a normal parathyroid hormone level. A skin biopsy specimen showed necrotic skin and subcutaneous fat with foci of calcification around the vessels, findings that confirmed the diagnosis of calciphylaxis. The patient then underwent subtotal parathyroidectomy and responded with initial improvement; however, her condition later deteriorated, and she died of uncontrolled pulmonary hemorrhage. CONCLUSION: Systemic calciphylaxis is a rare but devastating complication of end-stage renal disease. This condition mimics many different disorders; therefore, a high index of suspicion is essential for early recognition and diagnosis, which is assisted by biopsy specimens of skin lesions. Treatment includes subtotal or total parathyroidectomy in conjunction with autotransplantation, administration of phosphate binders, aggressive treatment with antibiotics, and lowering of the calcium-phosphate product. Even though a successful outcome is possible when this disease is recognized early and treated aggressively, the morbidity and mortality associated with systemic calciphylaxis still remain high.