Calciphylaxis: a conundrum for patients and nephrologists?

In 1961 Selye actively induced soft tissues calcification and necrotic eschars in rats using first “sensitizing agents” (injections of parathyroid glands extract, high dose vitamin D, high phosphate diets) and then “challenging agents” (local trauma or injection of egg white or ferric dextran). The resulting phenomenon, reproduced also in nephrectomised rats, was interpreted as hypersensitivity reaction and thus named “calciphylaxis” (from calcium and anaphylaxis) [1]. Years later, similar cutaneous lesions were described in patients with chronic renal failure (CRF) and, since then, a huge number of cases have been recorded. Nowadays calciphylaxis is defined as a condition of small arterioles calcification with eventual micro-vessels occlusion resulting in thrombus formation and tissue necrosis. As such, calciphylaxis is a rare (registered in orphan.net as ORPHA280062) and possibly underdiagnosed disease.