Calciphylaxis with normal renal and parathyroid function: not as rare as previously believed.

BACKGROUND: Calciphylaxis is a life-threatening form of metastatic calcification-induced microvascular occlusion syndrome. Although traditionally observed in patients with end-stage renal disease and/or hyperparathyroidism, the development of calciphylaxis in "nontraditional" patients having both normal renal and parathyroid function has been reported. However, to date there has been no collective analysis identifying common patient characteristics potentially predisposing to the development of calciphylaxis in nontraditional patients.

OBSERVATIONS: A 58-year-old woman with endometrial carcinoma developed extensive calciphylaxis despite the presence of normal renal and parathyroid function. The disease resolved with rapid diagnosis, supportive therapy, and medical management. Analysis of this case and the 13 previously reported cases of nontraditional calciphylaxis identified the following patient characteristics that highlight clinical situations potentially predisposing to calciphylaxis: hypoalbuminemia, malignant neoplasm, systemic corticosteroid use, anticoagulation with warfarin sodium or phenprocoumon, chemotherapy, systemic inflammation, hepatic cirrhosis, protein C or S deficiency, obesity, rapid weight loss, and infection.

CONCLUSIONS: Calciphylaxis is becoming increasingly common in patients with normal renal and parathyroid function. The observations from this study may assist dermatologists in the rapid diagnosis and prompt initiation of therapy for this devastating disease.