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What is calciphylaxis?

Photo: What is calciphylaxis?
Calciphylaxis falls into the class of ischemic wounds and occurs mostly in patients undergoing hemodialysis. It is a rare syndrome where blood vessels calcify losing their elasticity and initiate thrombosis through a series of inflammatory pathways. Eventually the thrombus (clot) obstructs the blood vessel and hence ischemia results preventing blood supply to the affected tissue.

Calciphylaxis results in serious wounds and is almost always fatal as the wounds fail to heal in this condition. It occurs mostly in patients in end stage renal disease and undergoing hemodialysis or have recently had a kidney transplant.

Cause

The cause of calciphylaxis is not entirely known. It has been established that this is not an immune type reaction such an allergic reaction. The current theory is that abnormal calcium and phosphate ion homeostasis results in excessive deposition of calcium in blood vessels. Following this deposition a tissue infarction occurs where the events leading to thrombosis are initiated.
Risk factors include:

  • Obesity
  • Diabetes
  • Female sex
  • Warfarin medication
  • Diagnosis

At present there is no particular diagnostic test for calciphylaxis. Diagnosis is made clinically through observing the nature of the skin lesions. These lesions are ischemic skin lesions with areas of necrosis where the skin is completely dying or dead. They are purplish or black in appearance with a leathery texture— and extremely painful. Furthermore these painful lesions cover a large area of skin. Diagnosis can be confirmed by conducting a skin biopsy.

Treatment

As calciphylaxis is almost always fatal the best mode of treatment is prevention. This involves of careful control of phosphate and calcium levels to prevent the metabolic changes that lead to calciphylaxis. However it is still uncertain whether the levels of calcium and phosphate are the main contributing factors in the development of calciphylaxis. There is no internationally recognised mode of treatment for calciphylaxis.

Treatments attempted include:

  • Dialysis
  • Appropriate wound care
  • Pain management
  • Preventing local tissue trauma such as subcutaneous infections
  • Sodium thiosulfate

Pain management is the initial step as the patient is in extreme discomfort. This involves administering appropriate oral medications and applying a lidocaine containing hydrogel dressing on the wound.

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