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E83.59 – Other disorders of calcium metabolism
237900002 – Calciphylaxis
SynopsisCalciphylaxis is the diffuse deposition of insoluble calcium salts in the skin due to systemic dysregulation of calcium metabolism. Disorders of calcium metabolism can be broadly categorized into 4 main groups: dystrophic, metastatic, idiopathic, and iatrogenic. Calciphylaxis is the most severe form of metastatic calcification and is most commonly associated with chronic renal failure, patients on hemodialysis, and secondary hyperparathyroidism.
While the exact pathogenesis is unclear, characteristic pathologic findings include progressive medial calcification of cutaneous blood vessels and subsequent ischemic necrosis of the skin. In addition, the process is believed to be triggered by chronic hypocalcemia from decreased intestinal absorption of calcium; this leads to increased levels of parathyroid hormone (PTH) and subsequent recruitment of calcium and phosphate from bone.
Painful, violaceous patches are initially seen clinically, followed by necrosis, ulcers, and/or gangrene. Mortality from calciphylaxis is high (60%-87%) and is secondary to sepsis from large, nonhealing ulcers.
Risks for developing calciphylaxis include female sex, diabetes, obesity, and calcium-based phosphate binders. The condition has also been observed in patients with breast cancer treated with chemotherapy, systemic lupus erythematosus, end-stage liver disease, and Crohn's disease.