Calciphylaxis (kal-sih-fuh-LACK-sis) is a serious, uncommon disease in which calcium accumulates in small blood vessels of the fat and skin tissues. People who have this condition usually have kidney failure and are on dialysis or recently had a kidney transplant. Calciphylaxis causes painful skin ulcers and may cause serious infections that can lead to death.

  • Expertise and experience. Even though calciphylaxis is uncommon, Mayo Clinic doctors have the expertise and experience to diagnose and treat this disease.
  • Multispecialty team. Calciphylaxis is a complicated disease that can affect different organs. Mayo's specialists in nephrology, dermatology, plastic surgery, endocrinology, infectious disease and other areas as needed work together to treat people with calciphylaxis.
  • Cutting-edge treatment. Mayo Clinic doctors use a three-pronged approach to treat calciphylaxis that includes decreasing calcium accumulation, restoring blood flow and intensive wound treatment. Mayo Clinic dermatology pioneered the use of low-dose tissue plasminogen activator (TPA) to dissolve tiny blood clots in calciphylaxis.

About

Calciphylaxis is a serious condition in which abnormal amounts of calcium are deposited in the body, mainly in the small blood vessels that supply fat tissue (vascular calcification). The cause of calciphylaxis is unknown, but doctors believe that an imbalance in the metabolism of calcium causes calcium to be deposited in the smallest parts of the arteries (arterioles). This eventually leads to the formation of blood clots in the arterioles, depriving the fat and skin above it from oxygen and nourishment.

Calciphylaxis most commonly affects people who have end-stage kidney failure. Risk factors include:

  • Long-term dialysis and sometimes kidney transplantation
  • An imbalance of calcium, phosphorus and aluminum in the body
  • Vitamin D therapy, which can increase the buildup of calcium and phosphorus
  • Some medications, such as calcium-binding agents or corticosteroids
  • An overproduction of parathyroid hormone (PTH), which regulates the level of calcium and phosphorus in the body — a condition known as hyperparathyroidism
  • Uremia, a toxic condition in which substances normally eliminated in the urine — such as calcium and phosphorus — build up in the body

Rarely, calciphylaxis occurs in people with no known kidney or calcium abnormalities for unknown reasons.

If you have this disease, you will often have:

  • Deep, very painful lumps with open sores and brown crust that fail to heal — typically in skin areas with high fat content, such as the stomach and thigh, although they can occur anywhere
  • Infections from wounds that don't heal

Complications of calciphylaxis include:

  • Pain and ulceration
  • Blood infections
  • Death, usually due to infection and multiorgan failure

Typically, the outlook for people with calciphylaxis is not good, so early detection and treatment is very important.

Mayo Clinic in Rochester, Minn., ranks #1 for kidney disorders in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Scottsdale, Ariz., is ranked among the Best Hospitals for kidney disorders, and Mayo Clinic in Jacksonville, Fla., is ranked high performing for kidney disorders by U.S. News & World Report.

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To determine if you have calciphylaxis, your doctor will review your medical history, assess your symptoms and do a physical exam. Tests may include:

  • Deep skin biopsy. To definitively diagnose calciphylaxis, your doctor may remove a small tissue sample from an area of affected skin for analysis.
  • Blood tests. Mayo Clinic labs will analyze a blood sample to measure a variety of substances in your blood — calcium, phosphorus, parathyroid hormone, aluminum, urea nitrogen, creatinine and albumin, among others — to help your doctor assess your kidney and liver function.
  • Imaging studies. X-rays may show the branch-like vascular calcifications that are common in calciphylaxis. However, this is also a common symptom in other advanced kidney diseases. Additional imaging exams, such as high-resolution CT scans, mammograms or bone scans may support the diagnosis of calciphylaxis.

Read more about blood tests for urea nitrogen creatinine and liver function tests.

No standard or universally effective treatment for calciphylaxis exists. Early diagnosis and treatment offer the best chance to reduce the effects. At Mayo Clinic, calciphylaxis is treated using three approaches explained below.

Intensive wound treatment

Your doctor will recommend aggressive wound care. For sores to heal, some of the tissue damaged by calciphylaxis may need to be surgically removed (debridement). In some cases, tissue can be removed using other methods, such as wet dressings or whirlpool treatments, among others. Antibiotic treatment is part of treating and preventing wound infection. You may be offered medication to assist with pain due to the calciphylaxis or wound care.

Restoring oxygen and blood flow to the skin

Your doctor may recommend hyperbaric oxygen therapy to increase oxygen delivery to the affected parts of the body. Also, your doctor may use low-dose tissue plasminogen activator (TPA) to dissolve the blood clots in tiny blood vessels of the skin. Medication that thins your blood may be prescribed to prevent additional blood clots from forming.

Decreasing calcium deposits

Reducing calcium deposits in your arteries may be helped by:

  • Dialysis. Your doctor may change your dialysis prescription if you're on kidney dialysis, including medications used in and frequency of your dialysis.
  • Medications to reduce calcium. Your doctor will evaluate your current medications and eliminate potential triggers for calciphylaxis, such as corticosteroids or iron, and may modify the amount or dose of your calcium or vitamin D supplements. Your doctor may also recommend a medication called cinacalcet (Sensipar), which can help control parathyroid hormone (PTH). Other medications may include sodium thiosulfate, which binds with calcium so it passes out of the body in urine, and medications to improve the balance of calcium and phosphorus.
  • Surgery. If your doctor determines that an overactive parathyroid gland (hyperparathyroidism) is contributing to the abnormal calcium metabolism by producing too much PTH, surgery to remove all or part of the parathyroid glands may be recommended.

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

At Mayo Clinic in Arizona, a team of specialists in dermatology, nephrology and other areas treats adults who have calciphylaxis.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic in Florida, a team of specialists in dermatology, nephrology and other areas treats adults who have calciphylaxis.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic in Minnesota, a team of specialists in dermatology, nephrology and other areas treats children and adults who have calciphylaxis.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Researchers at Mayo Clinic have been involved in a variety of studies focused on prevention, risk factors, causes and treatment of calciphylaxis.

Publications

See a list of publications by Mayo doctors about calciphylaxis on PubMed, a service of the National Library of Medicine.

Apr. 12, 2014