Calciphylaxis cutis

Calciphylaxis cutis, also known as calcific uremic arteriolopathy (CUA), is a rare and life-threatening condition characterized by the calcification of small blood vessels in the skin and subcutaneous fat, leading to progressive and extremely painful skin necrosis. The disease primarily affects the skin and subcutaneous tissue, but the underlying vascular calcification reflects a systemic disturbance of calcium and phosphate metabolism. It is most commonly seen in patients with end-stage renal disease (ESRD) undergoing dialysis, though it can also occur in patients with earlier stages of chronic kidney disease or, rarely, in individuals with normal kidney function (non-uremic calciphylaxis). The hallmark clinical features include intensely painful, violaceous (purplish) mottled skin lesions that progress to firm, necrotic eschars and non-healing ulcers. These lesions most frequently appear on the lower extremities, abdomen, and thighs, particularly in areas with significant adipose tissue. Secondary bacterial infection of necrotic wounds is common and represents a major cause of sepsis and mortality. The condition carries a very high mortality rate, estimated at 60–80% within one year of diagnosis, primarily due to sepsis and multi-organ failure. Treatment is multidisciplinary and includes aggressive wound care, pain management, correction of calcium-phosphate imbalances, and discontinuation of contributing medications such as warfarin and calcium-based phosphate binders. Sodium thiosulfate, administered intravenously, has emerged as a key therapeutic agent, acting as a calcium chelator and antioxidant, though evidence is largely based on case series and observational studies. Intensification of dialysis, parathyroidectomy in cases of severe hyperparathyroidism, and hyperbaric oxygen therapy may also be considered. Early recognition and intervention are critical to improving outcomes.

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