Abstract
Calciphylaxis is a rare but fatal disorder characterized by systemic medial calcification of the small arteries and ischemia of the subcutaneous tissues, often leading to necrosis of the subcutaneous fat and skin. This condition most commonly affects patients with end-stage renal disease (ESRD) and the pathogenesis is poorly understood. At present, there is no established treatment. We recently encountered three cases of calciphylaxis. The first case involved a 66-year-old man with ESRD who had been maintained on peritoneal dialysis(PD) for 7 years. The patient was admitted with painful ulcers on his thighs, hip, waist and wrists. Skin biopsies demonstrated calciphylaxis. Treatment with bisphosphonate and sodium thiosulfate hydrate had no effect and he died of peritonitis and DIC soon after admission. The second case involved a 53-year-old woman with ESRD who had been maintained on hemodialysis for 2 years. The patient was admitted with painful purpura on the lower legs and diagnosed with calciphylaxis based on skin biopsies. Hyperbaric oxygen therapy was initiated, and showed prominent results with symptom resolution and subsequent discharge. The third case involved a 72-year-old man with ESRD maintained on PD for 2 years, who was admitted for treatment of pneumonia. He displayed painful purpura on the lower legs and glans, and skin biopsies demonstrated calciphylaxis. He died of peritonitis and DIC before treatment could be started. All three patients were receiving warfarin for atrial fibrillation and vitamin D for secondary hyperparathyroidism, while levels of serum calcium levels were normal. We postulated that warfarin use may play an important role in the pathogenesis of calciphylaxis.
