Calciphylaxis is a rare, painful and deadly disease that is most likely to be seen in people with moderate to severe kidney problems. It happens when causes calcium deposits form in your blood vessels and block blood flow to areas of your skin. This can lead to open wounds that are prone to dangerous (or even deadly) infections.
Calciphylaxis is a rare, painful and deadly disease that is most likely to happen in people with moderate to severe kidney problems. The disease causes calcium deposits to form in your blood vessels and block blood flow, leading to areas where skin and tissue just underneath break down and die.
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Calciphylaxis has two types based on when they happen.
Uremic calciphylaxis makes up the majority of cases and happens in people who are on dialysis because of 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 calciphylaxis is much less common. It can also happen to people who don't have any kidney disease or problems, but this is extremely rare.
Calciphylaxis is also more common in women, who are twice as likely to develop the condition as men.
Calciphylaxis is very rare. In people who are on dialysis, it happens to approximately 35 of every 10,000 people (or about one-third of 1%). In the United States, there are just over 550,000 people on dialysis, so just under 2,000 cases of calciphylaxis happen on average each year. In countries with lower rates of kidney disease, calciphylaxis is much rarer.
In most cases, calciphylaxis happens because calcium deposits form in and block the blood vessels that supply your skin and the tissue just underneath it. They can form internally in the eyes, lungs, brain, muscles, and intestines in rare cases.
When it affects surface tissue like skin, calciphylaxis is extremely painful. It also causes the affected area to become an open wound, similar to a bedsore. The skin and tissue around that wound die, and the damage from the dead tissue spreads outward. These wounds are slow to heal and can lead to major complications.
Your skin is the most important part of your body’s defense against infection — as long as it’s intact —because it keeps germs out. When a wound gets infected and that infection spreads through your body, it can cause an overreaction of your immune system called sepsis. This condition is the most dangerous and deadliest complication of calciphylaxis.
When lesions happen, they most often happen in one of two ways:
These are lesions that happen mostly on the abdomen and thighs. They are more common in people who have a higher body mass index and in women.
At least 70% of lesions in uremic calciphylaxis cases have central distribution, while in non-uremic cases about half of the lesions have central distribution. Cases with more central lesions have a higher risk of death.
These are lesions near the ends of your arms and legs, especially on your fingers and toes. When peripheral lesions become advanced, this can lead to amputation to stop the damage from spreading and causing deadly complications.
The exact cause of calciphylaxis remains unknown, but the available research suggests it takes more than one factor to cause it. Researchers found that there were two requirements before calciphylaxis could happen.
In the case of calciphylaxis, hypersensitivity develops because of something that is happening to or around you. Many potential causes of hypersensitivity, known as sensitizers, have connections to each other. Sensitizers are like tinder material. They don’t cause the fire on their own. They just make it more likely to start.
Some of the possible sensitizers include:
Where sensitizers are like flammable tinder, triggers — sometimes called challengers or challenging agents — are the sparks that can start the calciphylaxis process. Possible triggers include:
Several conditions and risk factors might have connections to calciphylaxis, but none are proven causes. They include:
A medical professional, usually a doctor, can often begin to suspect calciphylaxis based on your condition, symptoms and a physical exam of your body. This includes looking and feeling for any changes to the skin or just underneath it. A healthcare provider will also ask questions about your medical history, personal circumstances and more as they try to make a diagnosis. Once a provider suspects calciphylaxis, the next step is confirming that suspicion with medical tests.
Because available research on calciphylaxis is limited,
The following tests are possible or likely:
Taking a sample of skin and tissue just underneath (usually about 4 or 5 millimeters deep) is the most reliable way to identify calciphylaxis positively. The sample is usually taken at the edge of a lesion or wound. Analyzing the sample involves looking at it under a microscope and using certain substances to cause color or chemical changes that can help confirm the diagnosis.
In cases where a person already has a diagnosis of end-stage kidney disease and has more advanced wounds with black scabbing, a biopsy may be unnecessary.
Blood and urine tests are often useful in helping with the diagnosis, assessing the severity of the case and how best to treat it. The tests used most often include:
Diagnostic imaging tests like X-rays or bone density scans may be done, but they aren’t as common. Using these tests is more likely when other tests are inconclusive.
Because there isn’t a great deal of research on calciphylaxis available, there’s also limited guidance on the best ways to treat it. Currently, the disease isn’t curable, but it can go into remission if treatment is successful.
In general, healthcare providers will focus on the following:
Preventing infection and helping wounds to heal are both top priorities. This can include several advanced types of care, such as:
Calciphylaxis is an extremely painful condition, making it harder for you to rest and let your body heal. Opioid pain medications tend to be the most recommended because morphine can cause low blood pressure, which is not ideal.
This medication, given in an IV, may help remove calcium built up in blood vessels or other tissues.
Ensuring the right levels and balance of minerals in your blood is important to help avoid complications and problems. 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 one or both of the parathyroid glands.
This therapy removes waste products from your blood when your kidneys aren’t working as they should. Managing this type of care also takes into account any concerns possible with calciphylaxis.
It's important to avoid malnourishment or dietary deficiencies that can happen with kidney problems and related conditions.
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 medications that are suspected sensitizers or triggers.
Your healthcare provider can best explain to you the possible complications and side effects that may happen. Because calciphylaxis isn't well-understood, complications and side effects can be unpredictable and may be very different from person to person.
Some of the possible side effects and complications include:
Following your healthcare provider’s instructions as closely as possible is very important with calciphylaxis. You should make sure to 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 healthcare provider about any changes to your diet, medication or any new supplements or home remedies you want to take.
Because the causes of calciphylaxis are not fully understood, it is harder to prevent it from happening. For that reason, healthcare providers often recommend minimizing use or exposure to sensitizers or triggers as much as possible. This includes things in your diet, personal care or hygiene items, medications, etc.
Unfortunately, the outlook for calciphylaxis tends to be negative because the disease isn’t well-understood. About half of all patients with this condition do not survive more than a year, with sepsis being the most common cause of death.
Depending on the circumstances of the condition, the one-year survival odds may be higher or lower. Those circumstances and odds are as follows:
Calciphylaxis is a chronic, life-long condition because it isn’t currently curable. However, it’s possible in some cases for the disease to go into remission after treatment. How long it’s possible to keep it in remission isn’t yet known.
If you think you might be or know you’re at risk for calciphylaxis, or if you already have a diagnosis of this condition, it’s a good idea to talk to your healthcare provider. They are the best source of information on your risk factors, what symptoms you should watch for, and when you should seek medical attention.
In general, you should pay attention to your body. For example, if you notice new spots that are painful, changes in the color of your skin, or if your skin or the tissue underneath feels different, you should call your provider. This can help them catch calciphylaxis early, which can be helpful in your treatment.
If you have calciphylaxis, your healthcare provider can also guide you when to call their office or seek immediate medical care in connection with 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:
A note from Cleveland Clinic
Calciphylaxis is a rare condition, and understanding it can be difficult because there’s limited available research and information. Talking to your healthcare provider or your loved one can help you better understand and know what to expect. While calciphylaxis may be a complicated and difficult-to-treat condition, some of the new research that’s now available may offer care options that can help.
Last reviewed by a Cleveland Clinic medical professional on 01/31/2022.
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