Calciphylaxis

Calciphylaxis (pronounced “kal-si-fuh-lack-sis”) is a rare, painful and often fatal disease that causes calcium deposits to form in your blood vessels and block blood flow. This leads to areas where skin and tissue just underneath break down and die.

In most cases, calcium deposits form in blood vessels that supply your skin and the tissue just underneath it. In rare cases, deposits can form in your eyes, lungs, brain, muscles and intestines.

When it affects surface tissue like skin, calciphylaxis is extremely painful. It also causes the affected area to become an open wound. The skin and tissue around that wound die, and the damage from the dead tissue spreads. These wounds are slow to heal and can lead to major complications.

Infection in a wound can spread through your body and cause an overreaction of your immune system called sepsis. This condition is the most dangerous complication of calciphylaxis.

In the U.S., calciphylaxis happens to about 35 of every 10,000 people who are on dialysis.

This condition is more common in females. They’re twice as likely to develop the condition as males. The average age at diagnosis is 50 to 70.

Types of this condition

Calciphylaxis has two types based on when they happen:

• Uremic (pronounced “you-reem-ick”): This type is the more common of the two. This typically happens in people who have end-stage kidney disease (ESKD). That means they’re in the later stages of kidney failure and need dialysis to filter waste from their blood.
• Non-uremic: This type is much less common. It happens in people who have earlier stages of kidney disease, who have a recent injury to one or both kidneys or who don’t have kidney problems at all (rarely).

Symptoms of calciphylaxis

Calciphylaxis symptoms may include:

• Very painful skin lesions (pain is worse than it appears, especially with touch)
• Vision problems
• Internal bleeding
• Muscle damage

Stages of calciphylaxis

Your experience of calciphylaxis changes with time. Researchers have found differences between early-stage and late-stage symptoms.

Early stage

Pain starts out changing between mild and severe. The feeling of pain may start before lesions or any other visible signs appear.

Areas of skin turn red, purple, brown or black. These lesions are on your belly and thighs or on your fingers and toes. Lesions usually have a netlike look. They may also blister, and the affected area of skin may become hardened and leathery. When calciphylaxis is due to cancer, lesions are typically firm, whitish nodules.

Late stage

Pain remains severe. The affected areas can also become very sensitive to pressure or touch. This makes the pain more intense.

Lesions usually become wounds that spread outward in starlike shapes. The wounds often have a foul smell. Areas of dead skin and flesh may turn black, look like scabs and fall off.

Calciphylaxis causes

The exact cause of calciphylaxis is unknown. But research suggests it takes more than one factor to cause it. These factors, which include imbalances of certain substances, can team up to cause this condition.

These factors include:

• Too much parathyroid hormone: When your parathyroid glands try to keep the calcium levels in your blood stable, they may make too much of a hormone your body needs. This can also happen when you aren’t getting enough vitamin D or calcium.
• Kidney failure: Kidneys that aren’t working properly disrupt your body’s chemical balance for handling calcium and phosphorus.
• Too much phosphorus: Your body uses phosphorus in several chemical processes, and your kidneys help control its levels.
• Certain types of cancer: This is more common with cases of calciphylaxis that have no link to kidney disease. Any cancer that destroys bone (lymphoma, leukemia, multiple myeloma and metastatic carcinoma) can cause calcium deposits in your blood vessels.
• Trauma: This can be due to either injuries or medical procedures, like an incision or a needle stick for an IV line.
• Ingestion of calcium: Excess ingestion of calcium (usually via supplements) will also lead to the same abnormal levels of calcium in your blood.

Risk factors

Several conditions and risk factors may be linked to calciphylaxis, including:

• Kidney problems: Most people who develop calciphylaxis are in the final stages of kidney failure and are on dialysis.
• Obesity: This condition increases your risk of developing calciphylaxis.
• Diabetes: Getting insulin injections every day may harm your skin at the injection site(s).
• Autoimmune diseases: Examples of these conditions include lupus, ulcerative colitis and rheumatoid arthritis.
• Liver disease: Alcohol-induced hepatitis, which is liver inflammation or disease from alcohol use, is a possible risk factor.
• Warfarin: This blood thinner blocks your body’s use of vitamin K, which has a role in blood clotting. Vitamin K also activates a protein that keeps calcification in check. Without enough vitamin K, calcium deposits can form more easily.
• Corticosteroids: These drugs treat swelling and inflammation. People who develop calciphylaxis have often taken corticosteroids recently.

How to lower your risk

Having longer or more frequent dialysis sessions may help remove calcium and other substances. But you can also adjust what you eat so you take in less calcium or phosphate. Your healthcare provider may ask you to switch from warfarin to a different blood thinner. They’ll choose one that doesn’t affect your body’s use of vitamin K.

Complications of this condition

Complications of calciphylaxis may include:

• Insomnia
• Loss of appetite
• Depression
• Severe ulcers
• Infections
• Amputation
• Sepsis

How doctors diagnose this condition

A healthcare provider may suspect calciphylaxis based on your condition, symptoms and a physical exam. This exam includes looking and feeling for any changes to your skin or the area just under it. They’ll also ask questions about your medical history as they try to make a calciphylaxis diagnosis. Once a provider suspects the condition, they’ll order medical tests to learn more.

A healthcare provider may order the following tests:

• Skin biopsy: Taking a sample of skin and the tissue just under it is the most reliable way to identify this condition. A provider looks at the sample under a microscope. Certain substances cause color or chemical changes that can help confirm the diagnosis.
• Lab testing: Blood and pee (urine) tests can help with making a diagnosis, judging the severity of the case and deciding how best to treat it. Providers most often check kidney and liver function, how well your blood clots and whether your body is fighting an infection.
• Imaging: A provider may order diagnostic imaging tests, like X-rays or bone density scans, but it’s not as common. You’re more likely to have imaging when other tests don’t provide answers that help with a diagnosis.

How is it treated?

Researchers don’t fully understand calciphylaxis. So, there aren’t many clear-cut guidelines on the best calciphylaxis treatment. Currently, the disease isn’t curable. But your symptoms can go away if treatment is successful. Treatments include wound care and pain management, nutrition and medications.

In general, healthcare providers will focus on:

• Wound care: Preventing infection with antibiotics and helping wounds heal are both top priorities. You may receive several types of care, like hyperbaric oxygen therapy, removing dead tissue from a wound and using skin grafts from elsewhere in your body.
• Pain management: Calciphylaxis is an extremely painful condition, making it harder for you to rest and let your body heal. Providers prescribe strong pain medications for calciphylaxis.
• Sodium thiosulfate: This medication, which you get through an IV, may help remove calcium built up in blood vessels or other tissues.
• Treatment of bone and mineral problems: Ensuring the right levels and balance of minerals in your blood can help you avoid complications. This also includes making sure your parathyroid hormone levels don’t go too low or too high. In some cases, it may involve surgery to remove a gland that makes this hormone.
• Dialysis: This therapy removes waste products from your blood when your kidneys aren’t working as they should.
• Nutrition management: It’s important to avoid a lack of nutrients, which can happen with kidney problems and related conditions.
• Medication management: A review of all medications you’re taking is important to avoid possible side effects and problems. This is especially true if you take warfarin or certain other drugs that may raise your risk of calciphylaxis.

When should I see my healthcare provider?

Talk to your healthcare provider if:

• You think you might be or know you’re at risk for calciphylaxis.
• You already have a diagnosis of this condition.
• You notice new spots that are painful.
• You see changes in the color of your skin.
• Your skin or the tissue underneath feels different.

Your provider is the best source of information on your risk factors, what symptoms you should watch for and when you should seek medical attention. They can catch calciphylaxis early, which can be helpful in your treatment.

If you have calciphylaxis, your healthcare provider can guide you on when to call their office or seek immediate medical care for lesions or wounds. Among the biggest things to watch for are signs of infection or problems in and around a wound. These include:

• Swelling or redness around the wound
• An area around the wound that’s hot to the touch
• Fluid and/or a foul smell coming from a wound
• A black scab or covering or forming on the wound

Questions to consider asking your healthcare provider may include:

• What type of calciphylaxis do I have?
• What are the best treatments for me?
• Are there resources that can help me?
• What’s the prognosis for someone in my situation?

What can I expect if I have calciphylaxis?

The outlook for calciphylaxis tends to be poor because of a lack of understanding of the disease. Most people with this condition can’t get around well. They spend much of their time in a wheelchair or bed.

Following your healthcare provider’s instructions as closely as possible is very important with calciphylaxis. You should be especially careful when it comes to wound care, keeping any sores or ulcers clean and protected from infection. You should also consult with your provider about any changes to the foods you eat, medication you take, or any new supplements or home remedies you want to try.

Up to 8 out of 10 people with calciphylaxis don’t survive more than a year. Sepsis is the most common cause of death.

Depending on several factors, the one-year survival odds may be higher or lower. Those factors are:

• Non-uremic calciphylaxis: People who have calciphylaxis without end-stage kidney disease are less likely to die within a year.
• Ulcers and wounds: Lesions become ulcers and wounds in the late stages of calciphylaxis. The later a diagnosis happens, the worse the prognosis tends to be.
• Debridement of wounds: There’s evidence that removing dead or dying tissue improves survival.

How long calciphylaxis lasts

Calciphylaxis is a chronic, lifelong condition because it currently isn’t curable. But it’s possible, in some cases, for the disease to go into remission after treatment. This means your symptoms get better or go away. Researchers don’t know how long it’s possible to keep it in remission.