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Calciphylaxis

Overview

Calciphylaxis is a rare but serious condition. It happens when calcium builds up in the tiny blood vessels that run through the fat in your skin and tissues. Imagine tiny pipes carrying blood getting clogged with calcium deposits. This can cause problems throughout the body.

One of the main signs of calciphylaxis is the formation of blood clots. You might also feel lumps under your skin, and painful, open sores (called ulcers). These ulcers are a major concern because they can easily get infected. If an infection sets in, it can become a life-threatening situation.

Doctors don't fully understand what causes calciphylaxis. However, people who develop this condition often have kidney problems. When the kidneys aren't working properly, they can't filter waste from the blood as they should. This often leads to the need for treatments like dialysis (a machine that filters blood) or a kidney transplant. Importantly, calciphylaxis can sometimes occur even in people who don't have kidney disease.

Fortunately, there are treatments for calciphylaxis. These treatments can include different types of medications, medical procedures, and sometimes even surgery. The goal of treatment is to prevent blood clots and infections, reduce the buildup of calcium, help sores heal, and ease any pain. Doctors work to manage the condition and improve the patient's quality of life.

Symptoms

Calciphylaxis is a condition that causes problems with the skin. Here are some of the common signs:

  • Skin changes: You might notice unusual, net-like patterns on your skin. These patterns can be a purplish-pink color.

  • Painful lumps and sores: The condition often leads to the development of deep, painful lumps under the skin. These lumps can become open sores (ulcers). These ulcers often have a dark, black or brown crust, and they don't heal easily on their own. These sores are more likely to appear in areas with a lot of fat, like the belly, thighs, buttocks, and breasts. However, they can develop anywhere on the body.

  • Possible infections: Since the ulcers don't heal easily, they can become infected. This is a serious complication that needs medical attention.

Causes

Calciphylaxis is a condition where calcium builds up in the tiny blood vessels of fat tissue and skin. Doctors don't fully understand why this happens.

A significant number of people with calciphylaxis also have kidney failure or are on dialysis. Scientists don't know for sure why these conditions increase the risk.

Sometimes, the calcium buildup is connected to the parathyroid glands, small glands in the neck that control calcium levels in the body. If these glands produce too much parathyroid hormone, it can lead to calcium buildup. However, this isn't the cause in every case. Many people with overactive parathyroid glands don't develop calciphylaxis, and many people with calciphylaxis and kidney failure don't have overactive parathyroid glands. So the link isn't completely clear.

Several other factors likely contribute to calciphylaxis:

  • Increased blood clotting: When blood clots form more easily, it can cut off the supply of oxygen and nutrients to the fat tissue and skin, potentially leading to damage.

  • Reduced blood flow: Narrowed or blocked tiny arteries can restrict blood flow to the affected areas, causing painful lumps and open sores (ulcers) on the skin.

  • Tissue scarring (fibrosis): Thickening and scarring of tissues can further damage the affected areas.

  • Damage to blood vessel lining: The lining of the blood vessels (endothelium) can become damaged, which can affect blood flow and potentially lead to clots.

  • Inflammation: The body's natural response to injury or infection, inflammation, might play a role in the development or progression of calciphylaxis.

Risk factors

Calciphylaxis is a serious condition that often happens in people with kidney problems. It's not just kidney failure, though; several other things can increase your risk.

One risk factor is being a woman. Being overweight or obese also raises the chances. Having diabetes, or high blood sugar, can also make you more vulnerable. If your liver isn't working correctly, this can be a risk factor too.

Having had dialysis in the past is another risk factor. Dialysis is a treatment that helps remove waste and extra fluid from the blood when your kidneys can't do it on their own. A tendency for your blood to clot more easily (called a hypercoagulable state) is another risk.

Imbalances in your body's levels of calcium, phosphate, or a protein called albumin can also contribute to the risk of calciphylaxis. Certain medications, such as blood thinners like warfarin (Jantoven), drugs that bind calcium, and steroids (corticosteroids), can also increase the risk. These medications can sometimes affect the balance of minerals in your body.

Complications

Calciphylaxis can lead to serious problems. One of the main issues is intense pain. This pain can be severe and debilitating. The condition can also cause large, open sores (ulcers) on the skin. These ulcers are often very deep and won't heal on their own.

Another serious complication is the risk of blood infections (sepsis). Sepsis is a very dangerous response to an infection in the body. It can be life-threatening.

Unfortunately, calciphylaxis can also lead to organ failure, which can be fatal. In many cases, the condition is fatal because of infection or problems with the body's organs. Early detection and treatment of any infections are very important in preventing these complications. It's crucial to get medical attention quickly if you have any signs of infection or unusual skin sores.

Prevention

Calciphylaxis is a condition that's hard to prevent. However, if you're on dialysis or have kidney problems (like advanced chronic kidney disease), it's crucial to manage the amount of calcium and phosphorus in your blood.

Controlling phosphorus levels can be tricky. Your doctor might prescribe medicine to take with meals. You might also need to limit foods high in phosphorus. Following your doctor's instructions and attending all scheduled checkups is essential.

If you do develop calciphylaxis, your medical team will help you manage it. This could involve using special bandages on any sores or ulcers to stop infections. Daily cleaning of these areas is also important to prevent bacteria from growing.

Diagnosis

Figuring out if you have calciphylaxis starts with a visit to your doctor. They'll ask about your health history, your symptoms, and do a physical exam.

To get a better understanding, your doctor might recommend some tests:

  • Skin Biopsy: A small piece of skin from the affected area is taken and sent to a lab for examination. This helps see if there are signs of calciphylaxis in the skin tissue.

  • Blood Tests: Your blood is tested to check levels of various substances. These include creatinine, calcium, phosphorus, parathyroid hormone, and vitamin D. These tests are important because they help your doctor assess how well your kidneys are functioning. Problems with kidney function can contribute to calciphylaxis.

  • Imaging Tests: If the skin biopsy isn't definitive or can't be performed, imaging tests might be helpful. X-rays, for example, can show calcium deposits in your blood vessels. These deposits are common in calciphylaxis and other serious kidney conditions. The imaging can help pinpoint the location and extent of the calcium buildup.

Treatment

Calciphylaxis treatment relies heavily on proper wound care. Having a team of wound care experts is very beneficial. This team can help manage the sores and wounds that often develop during calciphylaxis.

Several approaches can help reduce calcium buildup in the arteries, a key factor in calciphylaxis:

  • Dialysis Adjustments: If you undergo dialysis, your doctor might adjust your dialysis schedule. This could involve increasing the number of sessions or the duration of each session. They might also change the medications used during dialysis. This is done to help remove excess calcium from the body.

  • Medication Changes: Your doctor will review all your medications to identify any that might be contributing to calciphylaxis. Certain medications, like warfarin, corticosteroids, and iron supplements, can sometimes trigger or worsen the condition. Your doctor might adjust the dosage of calcium or vitamin D supplements, or even stop you from taking them altogether.

  • Specific Medications: A medication called sodium thiosulfate can help lower calcium buildup. It's given intravenously (through a needle in a vein) three times a week, often during dialysis. Your doctor might also prescribe cinacalcet (Sensipar) to help control parathyroid hormone (PTH). Other medications may be used to help balance calcium and phosphorus levels in your body.

  • Surgery: If an overactive parathyroid gland is causing high PTH levels, surgery (called parathyroidectomy) might be needed. This surgery removes all or part of the parathyroid glands.

Treating the wounds themselves is also crucial. If the damage from calciphylaxis is significant, some of the affected tissue may need to be removed surgically. This is called debridement. Other methods, like wet dressings, can also be used to remove damaged tissue.

  • Infection Control: Antibiotics can be used to treat or prevent infections that develop in the wounds. These infections are common complications of calciphylaxis.

  • Pain Management: Pain is a frequent problem during calciphylaxis and wound care. Your doctor will likely prescribe pain medication. If opioid pain relievers are necessary, you may need to consult with a pain specialist.

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Disclaimer: August is a health information platform and its responses don't constitute medical advise. Always consult with a licenced medical professional near you before making any changes.

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