Calciphylaxis

Overview

Calciphylaxis (kal-sih-fuh-LAK-sis) is a rare, serious disease. It involves a buildup of calcium in small blood vessels of fat tissues and skin.

Calciphylaxis symptoms include blood clots, lumps under the skin and painful open sores called ulcers. If an ulcer becomes infected, it can be life-threatening.

The exact cause of calciphylaxis isn't clear. But people with the disease usually have kidney failure. That's a condition in which the kidneys no longer work as they should. Often, these same people also have received kidney failure treatments such as dialysis or a kidney transplant. Calciphylaxis can happen in people without kidney disease too.

Calciphylaxis treatments include various medicines, procedures and surgery. Treatment can help prevent blood clots and infections, reduce calcium buildups, heal sores, and ease pain.

Symptoms

Calciphylaxis symptoms include:

• Large netlike patterns on the skin that may look purple-pink in color.
• Deep, painful lumps in the skin that can become ulcers. The ulcers often have a black-brown crust that doesn't heal on its own. Ulcers tend to appear in areas with high fat content, such as the stomach, thighs, buttocks and breasts. But they can form anywhere.
• Infections from ulcers that don't heal.

Causes

The exact cause of calciphylaxis isn't known. The disease involves the buildup of calcium in the smallest parts of the arteries in fat tissues and skin.

Many people who get calciphylaxis also have kidney failure or receive dialysis. It's not known why people with kidney failure or people receiving dialysis are at higher risk of calciphylaxis.

For some people, the calcium buildup in calciphylaxis is linked with small organs in the neck called parathyroid glands. If the glands release too many parathyroid hormones, that can cause calcium to collect. But the link isn't clear. Most people with seriously overactive parathyroid glands don't get calciphylaxis. And many people with kidney failure and calciphylaxis don't have overactive parathyroid glands.

Other factors that seem to play roles in calciphylaxis include:

• A greater tendency for blood to clot. Blood clots can deprive fat tissues and skin of oxygen and nourishment.
• Reduced blood flow in small arteries, which can lead to skin lumps and ulcers.
• Thickening or scarring of tissue, also called fibrosis.
• Ongoing damage to the thin layer of cells that lines blood vessels. This also is called vascular endothelial injury.
• Swelling, called inflammation, in the body.

Risk factors

Calciphylaxis most often affects people who have kidney failure. Other risk factors include:

• Being born female.
• Obesity.
• Diabetes mellitus.
• Liver failure, when the liver stops working as it should.
• A history of dialysis. This procedure removes waste and extra fluid from the blood when the kidneys no longer can.
• A greater tendency for blood to clot, also called a hypercoagulable state.
• An imbalance in the body of the minerals calcium or phosphate, or of the protein albumin.
• Some medicines, such as warfarin (Jantoven), calcium-binding agents and corticosteroids.

Complications

Complications of calciphylaxis include:

• Serious pain.
• Large, deep ulcers that do not heal on their own.
• Blood infections.
• Death, mainly due to infection or organ failure.

Often, the outlook for people with calciphylaxis isn't hopeful. Finding and treating any infections early is key to preventing serious complications.

Prevention

There isn't a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it's important to keep blood levels of calcium and phosphorus under control.

Keeping blood levels of phosphorus under control often is a challenge. Your healthcare professional might have you take medicines with meals. You also may need to restrict certain foods that are high in phosphorus. It's very important to follow your healthcare professional's directions and go to all follow-up healthcare checkups.

If you have calciphylaxis, your healthcare team helps you prevent ulcer infections or other complications. You may need to apply special wound dressings or clean the ulcers daily to prevent germs called bacteria from growing.