Calciphylaxis is a syndrome that presents as small vessel vasculopathy and skin necrosis in afflicted individuals. It occurs most frequently in patients with renal failure. Age does not appear to be a factor as cases have been reported in patients aged six months to 83 years old.
Images of lesions and calcification from this disorder may be viewed on the Washington University Neuromuscular Disease Center website.
Those with renal failure have a higher risk for developing this condition when any of the following factors are present:
- Gender (female)
- Diabetes mellitus
The first year of dialysis treatment is the time period in which most cases appear in patients.
Symptoms of this disorder include lesions on the lower extremities and intense pain. Lesions may also appear on the torso or hand of the patient, though that occurs less often than lesions on the lower extremeties.
Diagnosis of this condition may be obtained by the use of blood tests that check creatinine levels, coagulation factors and to exclude vasculitis, among other tests; imaging studies including bone scintigraphy; or biopsy of a non-ulcerated lesion or subcutaneous tissue.
Several treatment options are used in the treatment of this condition. Some have been reported to show some success in patients. They most commonly used treatments include:
- Diet - may include phosphate restriction
- Intravenous sodium thiosulfate
- Hyperbaric oxygen
- Surgery – in extreme case to care for wounds and treat sepsis.
Patients usually have limited mobility and are hospitalized for treatment of this syndrome.
Complications range from moderate to death. They include gangrene, amputation, sepsis, non-healing lesions, organ failure and gastrointestinal hemorrhage.
Calciphylaxis is reported to have mortality rates as high as 60 - 80 percent. The survival rate is approximately 45 percent for one year and 35 percent for five years. Patients increase their odds when they carefully follow treatment recommendations during initial renal failure and when in treatment for this syndrome.
Calciphylaxis. WA Wilmer, SJS July 2003. University of California, San Francisco Medical Center.
Calciphylaxis: emerging concepts in prevention, diagnosis and treatment. Wilmer WA, Magro CM. May – Jun 2002. National Institutes of Health.