Calciphylaxis

A 61-year-old woman who was receiving dialysis for diabetes-associated end-stage renal disease was hospitalized for care of an abdominal wound that had been debrided and closed. At this time, the patient had several large, indurated, red plaques with central, stellate, black eschars on her abdomen, left buttock, and legs. An early focus of ulceration was noted superior to the stapled incision.

Histologic examination of a debrided tissue specimen established the diagnosis of calciphylaxis, a rare but life-threatening syndrome characterized by progressive ischemic necrosis affecting small and medium-sized arteries of the skin and subcutaneous tissue. It is often rapidly fatal because of the development of sepsis or progressive gangrene. The cause of calciphylaxis is poorly understood, but most cases are associated with chronic renal failure, usually with hyperparathyroidism or elevated serum levels of calcium and phosphorus. When these abnormalities are present, parathyroidectomy or normalization of the serum levels may prove curative. Further work-up revealed that this patient's serum levels of calcium, phosphorus, and parathyroid hormone were all normal.

Good local wound care is also of utmost importance. Hyperbaric oxygen therapy was reported to be successful for a patient whose condition worsened despite conventional measures, including subtotal parathyroidectomy.¹

The lesions of calciphylaxis are often dark red to violaceous and may be pruritic and tender; the abdomen, buttocks, and extremities are favored sites. The differential diagnosis includes vasculitis, pyoderma gangrenosum, and other forms of panniculitis. Drs Eric J. Lewis and Charles E. Crutchfield III of Minneapolis write that the patient described here was discharged after responding to the combination of aggressive debridement, antibiotic therapy, and optimization of hemodialysis parameters. Use of hyperbaric oxygen will be considered if a relapse occurs.

REFERENCE:
1. Fischer AH, Morris DJ. Pathogenesis of calciphylaxis: study of three cases with literature review. Hum Pathol. 1995;26:1055-1064.