By Mayo Clinic Staff
Calciphylaxis (kal-sih-fuh-LAK-sis) is a serious, uncommon disease in which calcium accumulates in small blood vessels of the fat and skin tissues. Calciphylaxis causes painful skin ulcers and may cause serious infections that can lead to death.
People who have calciphylaxis usually have kidney failure and are on dialysis or recently had a kidney transplant. The condition can also occur in people without kidney disease.
- 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 requires specialists in nephrology, dermatology, plastic surgery, endocrinology, infectious disease and other areas to work together to treat people with calciphylaxis.
- Multimodal treatment. Mayo Clinic doctors use a three-pronged approach to treat calciphylaxis that includes decreasing calcium accumulation, restoring blood flow and oxygenation to the tissues, and applying intensive wound treatment. Mayo Clinic dermatology pioneered the use of low-dose tissue plasminogen activator (TPA) to dissolve tiny blood clots in calciphylaxis.
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 tissues of oxygen and nourishment.
Calciphylaxis most commonly affects people who have end-stage kidney failure. Possible risk factors include:
- Being female
- Long-term dialysis and sometimes kidney transplantation
- An imbalance of calcium, phosphorus and aluminum in the body
- 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
- An abnormality in blood-clotting factors — substances in your blood that help stop bleeding and form blood clots
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 No. 1 for kidney disorders in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Scottsdale, Ariz., ranks among the Best Hospitals for kidney disorders by U.S. News & World Report. Mayo Clinic in Jacksonville, Fla., is ranked high performing for kidney disorders by U.S. News & World Report.
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 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 branch-like calcium deposits in the blood vessels (vascular calcifications) that are common in calciphylaxis. However, vascular calcifications are also common in other advanced kidney diseases.
Read more about the blood urea nitrogen (BUN test), creatinine test 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.
Applying 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 blood clots in the tiny blood vessels of the skin. Medication that helps keep your blood from clotting (anticoagulation medication) may be prescribed to restore blood flow to the tissues. Your doctor will go over with you the best anticoagulant for your case.
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 the medications used in and the 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 (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's campus in Arizona, a team of specialists in dermatology, nephrology and hypertension, 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.
- U.S. Patients
- International Patients
At Mayo Clinic's campus in Florida, a team of specialists in dermatology, nephrology and hypertension, 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.
- U.S. Patients
- International Patients
At Mayo Clinic's campus in Minnesota, a team of specialists in dermatology, nephrology and hypertension, 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.
- U.S. Patients
- International Patients
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.
See a list of publications by Mayo doctors about calciphylaxis on PubMed, a service of the National Library of Medicine.
June 18, 2015
- Santos PW, et al. Calciphylaxis (calcific uremic arteriolopathy). http://www.uptodate.com/home. Accessed May 7, 2015.
- AskMayoExpert. How is calciphylaxis treated? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- AskMayoExpert. What tests are used to diagnose calciphylaxis? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- Nigwekar SU, et al. Calciphylaxis: Risk factors, diagnosis, and treatment. American Journal of Kidney Diseases. In press. Accessed May 13, 2015.
- Wangen T, et al. Calciphylaxis: An unusual case with an unusual outcome. The American Journal of Nursing. 2014;114:24.
- Brandenburg VM, et al. Calciphylaxis in CKD and beyond. Nephrology Dialysis Transplantation. 2012;27:1314.
- Vedvyas C, et al. Calciphylaxis: A systematic review of existing and emerging therapies. Journal of the American Academy of Dermatology. 2012;67:e253.
- El-Azhary RA, et al. Retrospective analysis of tissue plasminogen activator as an adjuvant treatment for calciphylaxis. JAMA Dermatology. 2013;149:63.
- El-Azhary RA (expert opinion). Mayo Clinic, Rochester, Minn. June 6, 2015.
- Sluzevich JC (expert opinion). Mayo Clinic, Jacksonville, Fla. May 26, 2015.