Diagnosis and treatment of calciphylaxis

Calciphylaxis occurs due to calcium deposition in arterioles, which leads to ischemic ulceration of overlying skin. Two-year mortality rates from sepsis ranges from 50% to 80%. Calciphylaxis is most common in hyperparathyroidism secondary to chronic renal impairment and rarely occurs in the setting of normal renal function. Biopsy of the calciphylaxis ulcer reveals calcium deposits lining the vascular intima. Tissue calcification may also be seen on plain radiographs. Calcium-phosphate metabolism should be normalized by treating any underlying hyperparathyroidism with bisphosphonates, parathyroidectomy, and/or cinacalcet in addition to dialysis in chronic renal failure. Intravenous sodium thiosulfate has been used successfully to treat renal and normo-renal calciphylaxis. Sodium thiosulfate displaces calcium ions from calcium deposits to form calcium thiosulfate, which is excreted by the kidneys or dialyzed. Systemic glucocorticoids may prevent ulceration of early plaques of calciphylaxis. Hyperbaric oxygen, skin grafting, and iloprost infusions are useful adjuncts in the management of this debilitating condition.