What is Calciphylaxis? Diagnosis and Treatment

Written by Medicover Team and Medically Reviewed by Dr Girishma J , Dermatologists



Calciphylaxis is a rare but serious condition in which calcium builds up in the small blood vessels of the skin and fatty tissues, reducing blood flow and causing painful skin lesions, ulcers, and tissue damage. It most commonly occurs in people with advanced kidney disease or those receiving dialysis, although it can occasionally affect individuals without kidney disease. Symptoms include severe skin pain, purple or black skin patches, non-healing ulcers, and an increased risk of infection.

Diagnosis is based on clinical evaluation, blood tests, imaging studies, and sometimes a skin biopsy. Treatment focuses on wound care, pain management, correcting calcium and phosphate imbalances, treating the underlying condition, and preventing complications such as infection.


What are the Types of Calciphylaxis?

Calciphylaxis can manifest in various forms, each presenting distinct characteristics and outcomes.

  • Cutaneous Calciphylaxis: A rare and serious skin condition characterized by calcification of small blood vessels, leading to painful skin ulcers and tissue necrosis.
  • Vascular Calciphylaxis: Involves calcification of blood vessels throughout the body, resulting in reduced blood flow and tissue damage, commonly seen in patients with kidney disease.
  • Systemic Calciphylaxis: A severe form of the condition that affects multiple organs and tissues, leading to widespread calcification and organ dysfunction.
  • Gastrointestinal Calciphylaxis: Manifests as calcification of blood vessels in the gastrointestinal tract, causing severe abdominal pain, bleeding, and tissue damage.
  • Cardiac Calciphylaxis: Involves calcification of the coronary arteries and heart valves, leading to cardiovascular complications such as myocardial infarction and heart failure.

What are the Symptoms of Calciphylaxis?

Calciphylaxis typically presents with a range of symptoms affecting the skin and underlying tissues.

  • Skin lesions
  • Painful ulcers
  • Tissue necrosis
  • Black eschars
  • Skin discoloration
  • Skin inflammation
  • Fever
  • Malaise

What are the Causes of Calciphylaxis?

Calciphylaxis primarily occurs in patients with advanced kidney disease due to abnormal mineral metabolism leading to calcium deposits in small blood vessels.

  • Chronic kidney disease
  • Hyperparathyroidism
  • Diabetes
  • Obesity
  • History of vascular disease
  • Use of certain medications like warfarin
  • Vitamin D deficiency
  • High calcium or phosphate levels in the blood

When to See a Doctor for Calciphylaxis?

Seek medical attention if you notice painful skin lesions, purple or black patches, non-healing ulcers, or severe skin pain, especially if you have chronic kidney disease or are undergoing dialysis. Consult a Dermatologist as early diagnosis and treatment can help prevent serious complications.

Seek immediate medical care if you experience:

  • Rapidly worsening or extremely painful skin ulcers
  • Fever, chills, or signs of wound infection
  • Foul-smelling discharge from skin lesions
  • Blackening of the skin or tissue
  • Severe pain that is difficult to control

Prompt evaluation by a Dermatologist is essential to manage the condition, reduce complications, and improve healing.

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How is Calciphylaxis Diagnosed?

Calciphylaxis is typically diagnosed through a combination of clinical evaluation and various tests to assess the skin lesions and underlying tissue damage.

  • Skin biopsy
  • Blood tests
  • Imaging studies (such as Xrays or CT scans)

What are the Treatment Options for Calciphylaxis?

Calciphylaxis is typically managed through a combination of medical interventions aimed at addressing the underlying causes and symptoms of the condition.

  • Wound Care Management: Proper wound care with debridement, dressings, and infection control is essential in managing Calciphylaxis lesions to promote healing and prevent complications.
  • Pain Management: Medications like opioids, nonsteroidal antiinflammatory drugs (NSAIDs), and nerve blockers may be prescribed to help alleviate pain associated with Calciphylaxis.
  • Sodium Thiosulfate Therapy: Intravenous administration of sodium thiosulfate has shown promising results in treating Calciphylaxis by chelating calcium deposits and improving wound healing.
  • Parathyroidectomy: Surgical removal of the parathyroid gland may be considered in cases where hyperparathyroidism is contributing to the development of Calciphylaxis.
  • Hyperbaric Oxygen Therapy: Hyperbaric oxygen therapy involves breathing pure oxygen in a pressurized chamber and has been used as an adjunctive treatment to promote wound healing in Calciphylaxis patients.

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What are the Risk Factors of Calciphylaxis?

Calciphylaxis is more likely to occur in individuals with chronic kidney disease, diabetes, obesity, or those on certain medications like calcium-based phosphate binders.

  • Chronic kidney disease
  • Diabetes
  • Obesity
  • Hyperparathyroidism
  • Female gender
  • Caucasian race
  • Use of vitamin D analogs
  • Warfarin therapy

Frequently Asked Questions

1. What is calciphylaxis?

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare and serious condition where calcium deposits form in small blood vessels of the skin and soft tissues, leading to tissue death.

2. Who is at risk for developing calciphylaxis?

Individuals with endstage renal disease on dialysis or those with chronic kidney disease are at higher risk for developing calciphylaxis. Other risk factors include obesity, diabetes, and hyperparathyroidism.

3. What are the symptoms of calciphylaxis?

Symptoms of calciphylaxis include painful skin lesions that may appear as red or purple patches, nodules or ulcers. These lesions can progress rapidly and lead to severe complications if not treated promptly.

4. How is calciphylaxis diagnosed?

Diagnosis of calciphylaxis involves a physical exam, skin biopsy, imaging tests like Doppler ultrasound or CT scan, and blood tests to assess calcium and phosphate levels. It is important to rule out other conditions with similar symptoms.

5. What is the treatment for calciphylaxis?

Treatment for calciphylaxis focuses on wound care, pain management, addressing underlying medical conditions like kidney disease, and promoting wound healing. Therapies may include wound debridement, hyperbaric oxygen therapy, medications to control calcium and phosphate levels.

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