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What Is Calciphylaxis?

Medically Reviewed by Poonam Sachdev on August 24, 2022

Calciphylaxis is a rare disease that mostly affects people who suffer from kidney failure. If you have this disease, your body creates calcium deposits in your blood vessels. These deposits block proper blood flow to parts of your body, which can have serious effects.

On the surface, this condition can affect your skin function and appearance. Calciphylaxis can be characterized by large, purple, web-like markings on the skin. Another common sign is the appearance of large, brown, crusted sores. 

Calciphylaxis can also affect the function of your internal organs, though. 

Calciphylaxis Symptoms

Symptoms of calciphylaxis are distinctive. Most patients suffering from this disease will show these symptoms: 

  • Extreme pain. Patients may complain about pain in their skin before there are any visible signs of calciphylaxis. This is one of the first symptoms most people will experience. 
  • Purple or red skin markings. These markings will usually form a web or net-like pattern on the skin and be very painful. In the earlier stages, they will be purple or red. Later, they may change to brown or black. The texture of the skin can also change by becoming hard and leathery or blistered. 
  • Sores that don’t heal. The blister sites will turn into lesions on the skin. These sores develop black or brown crusts that are extremely painful. They will usually start to develop a star-shaped pattern and putrid smell. Crusted parts of the lesions tend to fall off, but the wound will continue to grow.
  • Vision problems. In some cases, these calcium deposits can make their way into the eyes and impair your vision.  
  • Internal organ damage. Calciphylaxis can affect your major internal organs. It can cause internal bleeding and tissue damage. 

Calciphylaxis Causes

The cause of calciphylaxis is currently unknown. Researchers are working to discover a reason why calciphylaxis happens by studying the biology and risk factors of the disease. 

Most people who get calciphylaxis are going through end-stage kidney failure. However, there are some other known factors that can put you at risk for calciphylaxis: 

  • Women have a higher risk. Women are twice as likely to develop the disease. 
  • Being overweight or obese. A high body mass index (BMI) can put you at risk for developing calciphylaxis. 
  • Issues with blood clotting. If your blood doesn’t clot normally due to natural factors or medication, it leaves you at a higher risk. 
  • History of kidney problems or transplant. This is a major risk factor. A history of kidney malfunction, dialysis, or kidney transplant can make you vulnerable to this disease. 
  • Trouble metabolizing calcium. A calcium imbalance or trouble metabolizing the mineral calcium can lead to this condition. 
  • Hormonal imbalance. Parathyroid hormone (PTH) regulates calcium levels in your body. If the balance of this hormone changes, it can put you at risk. 
  • Autoimmune disease. In rare cases, calciphylaxis isn’t caused by kidney problems. Sometimes, autoimmune diseases like Lupus, multiple sclerosis, and rheumatoid arthritis can lead to calciphylaxis. 

How Is Calciphylaxis Diagnosed?

If you or a loved one is suffering from these symptoms and is also suffering from kidney injury or kidney failure, your health care practitioner may suspect calciphylaxis. 

There are two types of calciphylaxis: 

Uremic. Uremic calciphylaxis is the most common type. People who get calciphylaxis when they have end-stage kidney problems are classified as uremic cases. 

Non-uremic. Non-uremic calciphylaxis is extremely rare. It can develop in people who merely have a kidney injury or who don’t have any kidney issues at all. It can also happen in people who are in the early stages of kidney disease. 

A series of tests can be performed to confirm a calciphylaxis diagnosis: 

  • Skin biopsy. A sample of skin, usually from a lesion or sore, is removed and studied under a microscope. Certain chemicals or reactive substances may be added to determine if it’s calciphylaxitisitic. In advanced cases, a biopsy might not be necessary because a visual inspection of the lesions is all that’s needed. 
  • Kidney function tests. If you are showing symptoms of calciphylaxis but don’t have a history of kidney problems, your healthcare provider may order tests to inspect how efficiently your kidneys are working. This is commonly done by testing a urine sample. 
  • Blood coagulation tests. These tests reveal if your blood clots and how long it takes. The results can help show if you’re at risk for internal bleeding and help guide a treatment plan. 
  • General blood tests. A blood sample will be taken and checked for minerals, hormones, and abnormalities. 
  • Liver function tests. The liver and kidneys are part of your body’s cleaning and filtration system. Liver tests can show doctors how efficiently your system is working. 
  • Imaging tests. Some imaging tests like X-rays could be used to diagnose buildup of calcium in the blood vessels.  

Calciphylaxis Treatment and Outlook

There isn’t currently a known cure for calciphylaxis. Doctors can help you manage the symptoms, though, by administering pain medication and doing things that encourage the wounds to heal. 

Your healthcare practitioner may also provide options that could reduce the calcium deposits in your body to stall the condition or relieve symptoms. That could include changing or introducing new medications, surgery, or dialysis.

Oxygen therapy is one way to encourage the wounds to heal. For this therapy, you’re placed inside an oxygen chamber where your wounds are exposed to 100% oxygen.

Wound debridement is a surgical procedure where a doctor will cut and remove dead tissue from around a lesion or wound. 

Lesions are either located centrally on the torso, back, and stomach areas, on the arms and legs, or both. In severe cases of tissue death, there’s a possibility of amputation. This is commonly performed on arms, legs, fingers, or toes. 

Calciphylaxis isn’t curable, but remission is possible. Approximately half of the people who get calciphylaxis die within a year of diagnosis. The chances of survival depend strongly on what stage the disease is in when it’s diagnosed. 

Another factor affecting survival rates is whether the calciphylaxis is uremic or non-uremic. Non-uremic patients tend to have a higher rate of survival. 

Show Sources

SOURCES: 

Cleveland Clinic: “Calciphylaxis.”

Mayo Clinic: Calciphylaxis.”

National Kidney Foundation: “Seeking a Treatment for Calciphylaxis.”

StatPearls: “Calciphylaxis.” 

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