Calciphylaxis (Calcific Uremic Arteriolopathy)

Abstract

Calciphylaxis is a syndrome of subcutaneous vascular calcification resulting in painful ulcers on the legs, thighs, or abdomen. The disease develops almost exclusively in patients with end-stage renal disease (ESRD) and is frequently fatal due to infectious complications. Calciphylaxis was described in 1962 by Hans Selye. He made rats hypercalcemic with “sensitizers” vitamin D or parathyroid hormone (PTH). “Challengers” were then administered. These included skin injury by hair-plucking or injections of various sorts. Injured areas calcified and became ulcerated. Injection of “challengers” intravenously resulted in systemic necrotic lesions associated with tissue calcification (Selye H. Calciphylaxis. Chicago: University of Chicago Press; 1962). The concept of calciphylaxis has been imperfectly applied to cutaneous ulcerations that develop in patients with end-stage renal disease due to vascular calcification and subsequent occlusion. While this clinical scenario has some parallels with Selye’s experiments, his experimental subjects were not uremic, and the calcifications were not vascular. Therefore, “calciphylaxis” does not accurately describe the syndrome. Because this disease does not conform to the model of calciphylaxis as described by Selye, many advocate eliminating that label in favor of “calcific uremic arteriolopathy.”