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Calciphylaxis - Symptoms & Treatment


Calciphylaxis is a fatal disease in patients due to calcification of cutaneous blood vessels. It is a poorly understood and highly morbid syndrome of vascular calcification and skin necrosis. It is a rarely occurring condition in which patients present with painful, violaceous, mottled lesions of the extremities and/or trunk that progress to skin and subcutaneous tissue necrosis, non-healing ulcers, and gangrene. It most commonly occurs in patients with end-stage renal disease who are on hemodialysis or who have recently received a renal transplant. It is a devastating disease with a high rate of mortality and morbidity. The disease produces purple or red lesions typically located on the lower extremities or abdomen. The lesions can then develop thick, dark crusts and open skin ulcers. It is seen mainly in patients with end stage kidney disease. Calciphylaxis is not uncommon. The pathogenesis of Calciphylaxis remains unclear as it differs from intima-based common atherosclerosis and extravascular calcium-phosphate deposits in organs such as the skin in Calcinosis cutis are not involved.

Calciphylaxis remains unclear as it differs from intima-based common atherosclerosis and extravascular calcium-phosphate deposits in organs such as the skin in Calcinosis cutis are not involved. Patients' movement is often hindered by these ulcerated lesions, which may cause intense pain. In cases of acute illness, patients are usually hospitalized in intensive care. Calciphylaxis is a rare, often fatal, systemic disorder characterized by deposition (precipitation) of calcium phosphate salts (calcification) in the medial layer of the arteries and soft tissues. Disorders that are most often implicated in the pathogenesis of calciphylaxis include chronic renal failure, hypercalcemia, hyperphosphatemia, an elevated calcium-phosphate product, and secondary hyperparathyroidism. The disease has a survival rate of one to five years, but, if detected and treated early in its course, the survival can be much longer. Calciphylaxis is more common in women than in men, in obese patients compared to those of normal weight, and in patients who have been taking corticosteroids or other immunosuppressive medicines.

Causes of Calciphylaxis

The common causes and risk factor's of Calciphylaxis include the following:

  • Calciphylaxis occurs in patients with end stage renal disease.
  • Infusion of medications such as iron dextran.
  • Recently received a renal transplant.
  • The use of immunosuppressive agents, especially corticosteroids.
  • It is associated with a condition known as secondary hyperparathyroidism.
  • Calciphylaxis can occur in those with high or normal levels of serum calcium and phosphate.
  • Long-term obesity.

Symptoms of Calciphylaxis

Some symptoms related to Calciphylaxis are as follows:

  • Calcification of small- and medium-sized arterioles.
  • Fluid buildup.
  • Small blood clots develop within blood vessels.
  • Weight loss.
  • Excruciatingly tender and extremely firm lesions.
  • Less commonly, lesions may present as either bullae or distinct subcutaneous, erythematous nodules suggestive of erythema nodosum.

Treatment of Calciphylaxis

Here is list of the methods for treating Calciphylaxis:

  • Calciphylaxis is first treated with conservative therapy such as changing diet, medications and the dialysis prescription (for patients already on dialysis).
  • Blood thinning medications.
  • If calcium and phosphate levels remain high, especially in the setting of hyperparathyroidism, intravenous use of a vitamin D analog may be beneficial.
  • Low-calcium bath dialysis.
  • After nonresponse to conventional therapy, treatment with intravenous sodium thiosulfate 3 times weekly may be beneficial.
  • Hyperbaric oxygen treatments.
  • For patients with clearcut evidence of overactive parathyroid glands, surgery to remove all or part of the parathyroid glands may be necessary. Surgery may also be needed to remove dead or contaminated tissue to avoid infection or amputation.

 

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