Understanding Calciphylaxis: Symptoms and Outlook

Medically Reviewed By Jenneh Rishe, RN
Was this helpful?
1

Calciphylaxis occurs when skin cells die due to a lack of blood supply. Symptoms include blisters, bleeding, and pain. It mainly affects people with severe kidney disease, and there is currently no cure.

Data suggests that calciphylaxis has a 1-year mortality rate of over 50%. This means more than half of all people who get the condition may die within a year of diagnosis. Other complications can include blood infection, multiorgan failure, and sepsis.

This article explains calciphylaxis causes, symptoms, and treatment. It also discusses the complications of the condition.

What is calciphylaxis? 

Bandaging a knee
Wera Rodsawang/Getty Images

Calciphylaxis causes painful, blood-filled lesions to form on the skin. It mainly affects people with end-stage kidney disease. 

Many cases of the condition can have fatal consequences. Other cases lead to nonhealing wounds and frequent hospitalizations. Sepsis is another common effect. It occurs when the body overreacts to an infection. 

Other names for calciphylaxis include “calcific uremic arteriolopathy” and “calcific vasculopathy.”

Is it rare? 

Calciphylaxis is a rare condition. 

It has an estimated 1% per year of people undergoing dialysis. 

Is it fatal?

Calciphylaxis is a potentially fatal condition with a 1-year mortality rate of over 50%.

This means more than half of all people with the condition may die within a year.

What are symptoms of calciphylaxis? 

Calciphylaxis lesions usually have the following features:

  • Location: Calciphylaxis patches most commonly form on the lower limbs. However, they can also appear on the trunk, abdomen, buttocks, or thighs. 
  • Color: Calciphylaxis often begins as a purple-colored patch or blotch. It also typically causes the skin to become dark.
  • Blistering: The patches typically turn into blood-filled blisters and then ulcers over time.
  • Distribution: The ulcers can grow deep into the skin and become extensive.
  • Pain: Extreme pain is common with calciphylaxis.
  • Other sensations: Severe burning and itching may also occur at the sites of the lesions.
  • Syndrome: Calciphylaxis can cause blue-toe syndrome, which causes muscle pain in the legs and severe pain in the foot.

Sometimes calciphylaxis can also affect the eyes, brain, penis, intestines, muscles, or lungs. 

How does it affect the skin?

Calciphylaxis skin lesions typically start as discolored patches. Over time, they blister and turn into open sores. They also cause the tissue surrounding the wound to die.

This can cause an odor and intense pain. It can also cause sepsis.

Sepsis is the main cause of death in people with calciphylaxis.

When should you see a doctor?

Contact your doctor if you have symptoms such as painful, blood-filled lesions appearing on the skin. This is especially true if you have kidney disease.

Your doctor will make a diagnosis and begin treatment.

What causes calciphylaxis?

Severe kidney disease can interfere with the kidneys’ ability to remove waste from the bloodstream. It can also cause calcium deposits to build up within the blood vessels.

This buildup can disrupt the blood supply to the skin and cause skin tissue to die. In some cases, it can also affect the lungs, brain, muscles, and other internal organs.

Calciphylaxis can also occur in people with standard kidney function. Experts use the term “non-uremic calciphylaxis” to describe this phenomenon.

Causes of non-uremic calciphylaxis include:

  • malignancy or the presence of cancerous cells in the body
  • alcohol-related liver disease 
  • connective tissue disease
  • diabetes mellitus

Other contributing factors include:

  • excess phosphate in the blood
  • reduced levels of vitamin D in the blood
  • excess parathyroid hormone, which is a hormone that tries to control calcium levels in your blood
  • sensitizer compounds, such as lead, iron, and aluminum

How do you treat calciphylaxis?

Calciphylaxis is not currently curable, but it is treatable.

Treatment focuses on addressing the underlying factor.

Methods include:

  • calcium and phosphate restricted diet to balance calcium and phosphate levels in the blood
  • dialysis with a lower dialysate calcium concentration to lower calcium levels 
  • cinacalcet tablets to reduce excess production of parathyroid hormone 
  • surgical removal of the parathyroid glands to reduce pain and promote ulcer healing
  • anticoagulants to control the formation of blood clots

Other treatments include:

  • special wound dressings to reduce odors 
  • surgical removal of dead skin cells to prevent spreading 
  • systemic antibiotics to treat infection 
  • intravenous infusions of sodium thiosulfate to remove excess calcium from the blood
  • doxycycline to prevent new lesions from forming

Adequate wound care and pain management are an integral part of treatment for calciphylaxis.

Your medical professional will provide as much comfort as possible.

What are risks factors of calciphylaxis?

Several factors can increase a person’s risk of calciphylaxis. They include:

  • being female 
  • being overweight
  • being white 
  • using medications, such as: 
    • iron therapy
    • calcium-based phosphate binders
    • corticosteroids
    • warfarin
    • activated vitamin D
  • having hypoalbuminemia, which refers to insufficient amounts of the protein albumin in the bloodstream
  • having autoimmune conditions, such as: 

Sex and gender exist on spectrums. This article will use the terms “female” and “women” when discussing people assigned female at birth to reflect language in source materials.

Learn more about the difference between sex and gender here.

What are complications of calciphylaxis?

People with calciphylaxis can develop blood infections and sepsis. They may also experience extensive ulcers and chronic pain.

Other complications include:

  • amputation of the limbs
  • multiorgan failure 
  • death

Early diagnosis and treatment may help prevent some complications.

How can you prevent calciphylaxis?

It is unclear if calciphylaxis is preventable or not. However, you can take some steps to lower your risk of chronic kidney failure.

They include:

  • eating a healthy diet including fresh fruit, vegetables, and whole grains
  • reducing your alcohol intake
  • avoiding smoking
  • getting enough sleep
  • learning how to manage stress
  • exercising regularly
  • seeking prompt treatment for diabetes, high blood pressure, and heart disease

What is the outlook for calciphylaxis?

Calciphylaxis has a 1-year mortality rate of over 50%. Death usually results from secondary infections and sepsis. 

Treatment can typically help manage symptoms.

Summary

Calciphylaxis causes painful, blood-filled lesions to form on the skin. It results when calcium deposits accumulate within the blood vessels. Symptoms include blisters, bleeding, and pain.

Most people with calciphylaxis get it from severe kidney disease. Other risk factors include a high body mass index (BMI), diabetes mellitus, and alcohol liver disease.

Treatment focuses on addressing the underlying factor.

Methods include a calcium and phosphate restricted diet, dialysis with a lower dialysate calcium concentration, and cinacalcet tablets. 

Seek early treatment if you have symptoms of calciphylaxis.

Was this helpful?
1
Medical Reviewer: Jenneh Rishe, RN
Last Review Date: 2022 Sep 26
View All Vascular Conditions Articles
THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
  1. Fares, I., et al. (2019). Calciphylaxis: Successful management of a rare complication of chronic kidney disease in two patients. https://www.hindawi.com/journals/crin/2019/1630613/
  2. John, C. J. (2019). Calciphylaxis: Diagnosis, pathogenesis, and treatment. https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx
  3. Larsen, F. (2021). Calciphylaxis. https://dermnetnz.org/topics/calciphylaxis
  4. Preventing chronic kidney disease. (2016). https://www.niddk.nih.gov/health-information/kidney-disease/chronic-kidney-disease-ckd/prevention
  5. Seeking a treatment for calciphylaxis. (n.d.). https://www.kidney.org/professionals/Sagar-Nigwekar-Seeking-Treatment-Calciphylaxis
  6. Westphal, S. G., et al. (2022). Calciphylaxis. https://www.ncbi.nlm.nih.gov/books/NBK519020/