Calciphylaxis in a chronic hemodialysis patient with protein S deficiency

Vascular calcifications are common in uremic patients whereas calciphylaxis is rare. We report the case of a 45-year-old woman on chronic hemodialysis since 1977. She had a subtotal parathyroidectomy in 1985, aortic and mitral valve replacement in 1986, and has been treated since then with nicoumalone. In June 1991, she presented with repeated, painful cutaneous necrosis suggesting panniculitis. A skin biopsy showed lobular panniculitis and evidence of calciphylaxis. There was an obvious biological hyperparathyroidism. Protein C functional level was in the normal range whereas protein S functional level was low, i.e. 42%. The patient underwent cervical surgery to remove two parathyroid glands, and daily hemodialysis sessions. Despite this treatment, cutaneous necrosis progressed with superinfection. A few weeks later, the patient died from a septic shock after a myocardic infarction. Necropsy was not performed.