Calciphylaxis

Overview

Mayo specialists are experienced in treating calciphylaxis, an uncommon kidney-related disease in which calcium accumulates in small blood vessels in the skin. This condition results in painful skin ulcers and can be associated with organ failure and serious infections. A team of doctors, including nephrologists, dermatologists, plastic surgeons and endocrine and infectious diseases specialists, work together to prevent major ulceration and reduce sickness and death associated with this disease.

Diagnosis

Patients with this condition usually have experienced kidney failure and have undergone kidney dialysis. 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. Diagnostic tools include blood tests, imaging studies and biopsies. Read more about calciphylaxis diagnosis.

Treatment Options

Treatment usually begins with dietary changes to reduce calcium and phosphorus in the blood, stopping calcium and vitamin D supplements, changes in phosphorous-binding medications, and (for dialysis patients) changes in the dialysis prescription. Patients may also be treated for pain, organ failure and infection. A new drug, tissue plasminogen activator (tPA, alteplase), has shown promise in preventing major ulcerations and increasing patient survival. For patients with overactive parathyroid glands, surgery or a medication may be needed to regulate the production of PTH hormone, which controls how the body uses calcium and phosphorus. In severe cases of skin ulceration, removal of dead tissue or amputation may be necessary. Read more about calciphylaxis treatment options.

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About Calciphylaxis

Calciphylaxis lesions develop suddenly and progress rapidly, with one or many on the lower extremities.

Calciphylaxis is a serious condition in which abnormal amounts of calcium are deposited in the body, primarily in vessels that supply blood to the skin. It can be likened to a heart attack of the skin; due to obstructed blood vessels, the skin is deprived of oxygen and nourishment. People with the disease have lesions (sores) that fail to heal. They also frequently suffer infections from non-healing wounds and can develop gastrointestinal hemorrhage, infarction or organ failure. In rare cases, vascular calcification can be reversed with aggressive management. 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.

This uncommon condition affects 1 percent to 4 percent of patients with kidney failure who've undergone kidney replacement therapy. It can occur in non-dialysis patients with kidney disease (for example, kidney transplant patients), and in patients with liver disease but without any kidney disease. The incidence of calciphylaxis has increased, possibly because of widespread use of vitamin D injections and iron treatments. It is most prevalent in white, middle-aged females. It is associated with uremia, a toxic condition affecting patients with kidney disease in which elements normally eliminated in the urine (e.g., calcium, phosphate) accumulate in the body. Doctors believe that patients with calciphylaxis have unique changes in the walls of their blood vessels which cause cells to take on the characteristics of bone cells and deposit calcium.