Description

Calciphylaxis (kal-sih-fuh-LAK-sis) is a rare severe disease. Involves a buildup of calcium in the small blood vessels of the fat tissue and the skin.

Calciphylaxis symptoms include blood clots, bumps under the skin, painful, open sores called ulcers. If an ulcer is infected, it can be deadly.

The exact cause of calciphylaxis is unclear. But people with the disease usually have renal failure. This is a condition in which the kidneys stop functioning as they should. Often, these same people have also received the kidney failure treatments, such as dialysis or a kidney transplant. Calciphylaxis can occur in people without kidney disease too.

Calciphylaxis treatment include several medications, procedures, and surgery. The treatment can help prevent blood clots and infections, reduce accumulations of calcium, heal wounds, and relieve the pain.

Symptoms

Calciphylaxis symptoms include:

  • Great netlike pattern on the skin that can be colored with a purple-pink color.
  • Deep, growths on the skin that can become ulcers. The ulcers tend to have a brownish-black color of the cortex that does not heal by itself. The ulcers usually appear in areas with high fat content, such as the stomach, thighs, buttocks, and breasts. But they can form in any part.
  • Infections of the ulcers that do not heal.

Causes

The exact cause of calciphylaxis is not known. The disease is the accumulation of calcium in the smaller parts of the arteries and fat in the skin and tissues.

Many people who get calciphylaxis also have renal impairment or receiving dialysis. It is not known why people with renal impairment or in patients receiving dialysis are at greater risk of calciphylaxis.

For some people, the accumulation of calcium in calciphylaxis is associated with small organs in the neck, called parathyroid glands. If the glands release too much parathyroid hormone, which can cause calcium to pick up. But the link is not clear. The majority of people with serious hyperfunction of the parathyroid glands do not get the calciphylaxis. And many people with renal failure and calciphylaxis do not have the hyperfunction of the parathyroid glands.

Other factors that appear to play a role in calciphylaxis include:

  • An increased tendency of the blood to clot. Blood clots can deprive the fat of the skin and tissue of oxygen and food.
  • The reduction of blood flow in the small arteries, which can lead to bumps on the skin, and ulcers.
  • Thickening or scarring of the tissue, also called fibrosis.
  • Ongoing damage to the thin layer of cells that lines the blood vessels. This is also called vascular endothelial injury.
  • Swelling, called the inflammation in the body.

Risk factors

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

  • To be born female.
  • Obesity.
  • Diabetes mellitus.
  • Liver failure, when the liver stops working as it should.
  • A history of dialysis. This procedure removes waste and excess fluid from the blood when the kidneys no longer can.
  • An increased tendency of blood clotting, also called a hypercoagulable state.
  • An imbalance in the body's minerals, calcium, or phosphate, or protein of the albumin.
  • Some medications, such as warfarin (Jantoven); calcium-binding agents and corticosteroids.

Complications

The complications of calciphylaxis include:

  • Severe pain.
  • Large, deep ulcers that do not heal on their own.
  • Infections of the blood.
  • Death, mainly due to infection or organ failure.

Often, the outlook for people with calciphylaxis, is not optimistic. Search and treatment of any infection early is the key to the prevention of serious complications.

Prevention

There is not a clear way to avoid calciphylaxis. But if you are on dialysis, or who have a low kidney function due to advanced chronic kidney disease, it is important to maintain blood levels of calcium and phosphorus under control.

Keep phosphorus levels under control is often a challenge. Your health care professional may have to take medicines with meals. You may also need to restrict certain foods that are high in phosphorus. It is very important to follow the directions of your health care professional's directions and go to all the health checks.

If you have calciphylaxis, your care team will help to prevent the ulcer infections or other complications. You may need to apply special wound dressings or clean ulcers daily to prevent germs called bacteria growth.

Diagnosis

The diagnosis is to find out if the calciphylaxis, is the cause of your symptoms. Your health care professional review of your health history, asks you about your symptoms, and gives a physical examination.

You may also need tests such as:

  • Skin biopsy. During this procedure, the healthcare provider removes a small sample of tissue from an affected area of the skin. Then, a laboratory check of the sample.
  • Blood tests. A laboratory can measure various substances in the blood. These include creatinine, calcium, phosphorus, parathyroid hormone, and vitamin D. the results help your healthcare team to check how well your kidneys are working.
  • Imaging tests. These may be useful if the results of the biopsy is not clear or if the biopsy can't be done. X-rays may show calcium deposits in the blood vessels. These accumulations are common in calciphylaxis and in other advanced renal disease.

Treatment

Often, more than one treatment is necessary for calciphylaxis. The treatments of restoration of oxygen and blood flow to the skin and to reduce the buildup of calcium.

The restoration of oxygen and blood flow to the skin

Medication that helps prevent blood from clotting can restore the flow of blood to the tissues affected. These blood-thinning medicines are called anticoagulants. If you do not help, an experimental treatment may be available that can be used to dissolve blood clots in the small blood vessels of the skin. This treatment is a type of medicine called a low-dose tissue plasminogen activator.

Another backup option of treatment involves breathing pure oxygen in a pressurized chamber. This is called hyperbaric oxygen therapy. Is not available on all parts. A small amount of research suggests that this treatment can help to control infection and limit the loss of tissue, whereas the main effect of the treatments.

Wound care is a crucial part of the calciphylaxis treatment. So it can be very helpful to have a team of specialists in the care of the wound.

The reduction of the accumulation of calcium

The reduction of the accumulation of calcium in the arteries can be helped by:

  • The dialysis. If you receive dialysis treatment kidney disease, your healthcare provider may change the medications that are used and how much time and frequency of dialysis. It could be useful to increase the number and the duration of the dialysis session.
  • Change medications. Your health care professional for reviews of their current medications, and it gets rid of the potential triggers of calciphylaxis. These factors include warfarin, corticosteroids, and iron. If you take calcium or vitamin D supplements, your health care professional can change the amount you take or have you stop taking them.
  • Taking the medicines. A medication called sodium thiosulphate can reduce the accumulation of calcium in the small arteries. It is given through a needle placed into a vein three times a week, usually during the dialysis. Your health care professional may recommend that you take a medication called cinacalcet (Sensipar), which may help to control parathyroid hormone (PTH). Other medications can be used to improve the balance of calcium and phosphorus in the body.
  • Surgery. If a hyperactivity of the parathyroid gland that causes the overproduction of PTH plays a role in your condition, surgery may be a treatment option. Surgery called a parathyroidectomy may remove all or part of the parathyroid glands.

The application of intensive treatment of the wound

To heal the wounds, some of the tissue damaged by the calciphylaxis may need to be removed with surgery. This is called debridement. Sometimes, the tissue can be removed by other methods, such as wet dressings. Medicines called antibiotics can combat infections caused by germs. Antibiotics can help treat and prevent ulcer infections.

The control of pain

Probably offer him medication to control the pain due to calciphylaxis or during wound care. A pain medicine specialist may be necessary if you are prescribed opioid analgesics.

Preparing for your appointment

You can start by seeing your health care professional. Or you may be referred to a skin doctor called to a dermatologist or a doctor of the kidneys called a nephrologist.

Some of the other health professionals who may become part of your health care team include:

  • A doctor of internal medicine, called an internist.
  • A surgeon.
  • A wound care specialist.
  • A pain management expert.

Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do beforehand. For example, you may be asked not to eat for a certain number of hours before some of the tests. This is called fasting. It is also a good idea to bring a family member or a friend with you. A person of confidence that you can help you remember all the information of your appointment.

It is also helpful to make a list of:

  • Its symptoms, including those that do not seem to be related to the reason for your appointment.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All medications, vitamins or supplements that you take, including any amount that you take.
  • Questions to ask your health care team.

For calciphylaxis, some basic questions to ask your health care team include:

  • What is likely causing my symptoms? There are other possible causes?
  • What tests do I need?
  • What treatment do you recommend it?
  • I have other health conditions. How can I best manage these conditions?
  • Do I have to change my diet?
  • What is the outlook for my health?
  • Are there brochures or other printed material I can have? What sites do you recommend?

What to expect from your doctor

Your healthcare provider will likely ask questions such as:

  • When did your symptoms begin?
  • The symptoms been continuous or come and go?
  • How bad are the symptoms?
  • Nothing seems to improve the symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Calciphylaxis