Medical Information
Niels Olson


Kidney & Bladder Health
Skin Care
Contributed byKrish Tangella MD, MBAAug 09, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Calcific Uremic Arteriolopathy (CUA)
  • CUA (Calcific Uremic Arteriolopathy)

What is Calciphylaxis? (Definition/Background Information)

  • Calciphylaxis is a rare and serious disorder wherein there is necrosis of small-to-medium sized blood vessels. It can occur with multiple conditions such as poorly-controlled diabetes, end-stage kidney disease, and hyperparathyroidism
  • In this condition, there are calcium deposits that form within the blood vessel walls. Due to this, skin lesions appear on the body (frequently on the abdomen, groin, and lower extremities) that are extremely painful
  • Middle-aged and older adults are generally affected. The skin turns red and then black due to necrosis (death) of skin tissue. Calciphylaxis can lead to severe infections and even cause sepsis, when blood is affected
  • The contributory factors for Calciphylaxis may include renal disease, uncontrolled diabetes, deficiency disorders of protein C and protein S, autoimmune conditions such as lupus and rheumatoid arthritis, and even chemotherapy treatment for breast cancer
  • Undertaking a treatment of the underlying condition causing Calciphylaxis is the first step towards treating it. Other measures include use of pain medication and surgery to remove dead skin
  • However, in spite of treatment, the condition progresses slowly and steadily. The prognosis of Calciphylaxis is typically poor; it can lead to fatalities

Who gets Calciphylaxis? (Age and Sex Distribution)

  • Calciphylaxis is an uncommon condition that is observed in middle-aged and elderly adults in the 40-80 year age group
  • Both male and female genders are generally affected. Some reports indicate that females are affected more than males, while others show an equal predilection for both
  • Individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Calciphylaxis? (Predisposing Factors)

Following are the risk factors for Calciphylaxis:

  • Kidney disease
  • Uncontrolled diabetes
  • Increased calcium levels in blood which can be caused by a variety of conditions (hypercalcemia)
  • Obesity
  • Protein C deficiency and protein S deficiency
  • Long-term steroid use
  • Use of blood thinners such as warfarin
  • Severe malnutrition
  • Breast cancer treatment with chemotherapy
  • Alcoholic liver cirrhosis
  • Autoimmune conditions such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Crohn’s disease

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Calciphylaxis? (Etiology)

  • Calciphylaxis is caused by calcium deposits on the blood vessels that make it hard and more susceptible to blockage (thrombosis)
  • The calcium deposition can occur due to many underlying conditions such as end-stage kidney disease, poorly-controlled diabetes, hyperparathyroidism, or autoimmune disorders

What are the Signs and Symptoms of Calciphylaxis?

The signs and symptoms of Calciphylaxis may include the following:

  • The formation of skin lesions that are very painful
  • Initially, the lesions are erythematous (red in color), and as the disease progresses, they become black due to necrosis (death of cell or tissue)
  • The skin lesions turn from red to black over a period of months
  • Common sites that are affected include the legs, buttocks, abdomen, and penis

Signs and symptoms of the underlying condition may be present.

How is Calciphylaxis Diagnosed?

A few diagnostic tests that are performed for Calciphylaxis may include:

  • A thorough physical examination and a complete medical history (including history of diabetes, kidney disease, or autoimmune conditions) are very crucial; a dermatologist will carefully examine the skin to aid in the diagnosis
  • Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Blood tests that include the following:
    • Calcium levels
    • Blood phosphate
    • Serum parathormone levels
    • Blood glucose levels
    • BUN (blood urea nitrogen)
    • Blood creatinine
    • Blood culture
  • Imaging studies such as ultrasound, CT, or MRI scans
  • Vascular angiogram
  • Skin culture for bacteria, fungus, or virus
  • Potassium hydroxide (KOH) test: The KOH prep involves microscopic observation of skin scrapings to rule-out any skin fungal infections
  • Skin biopsy: A biopsy of the lesion is usually performed to confirm the diagnosis. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Calciphylaxis?

The complications of Calciphylaxis may include:

  • Necrosis of skin, fingers, or penis
  • Fungal, bacterial, or viral infections on the skin surface, due to bleeding and ulceration of the skin lesions
  • Secondary infections can cause sepsis
  • Individuals may have emotional and psychological stress from cosmetic issues
  • Severe cases may cause heart failure due to extensive calcification in the entire heart - a condition termed as “heart of stone”
  • Complications that may arise from the underlying cause of Calciphylaxis

How is Calciphylaxis Treated?

The main focus of Calciphylaxis treatment involves treating the underlying condition. The following measures may be considered:

  • Treating kidney failure, which may be through dialysis
  • Sometimes, immunosuppression medication in kidney transplant patients can result in Calciphylaxis. In such cases, reducing the dosage of immunosuppressants may be helpful
  • Hyperparathyroidism can be treated by performing partial thyroidectomy
  • Surgical removal of necrotic (dead) skin
  • Administration of pain medication
  • Use of topical steroidal creams and lotions
  • Hyperbaric oxygen therapy to improve wound ulcer healing
  • Antibiotics may be used to treat infections
  • Ensure proper skin care by avoiding the tendency to itch
  • Maintaining cleanliness and proper body hygiene

How can Calciphylaxis be Prevented?

Currently, it may not be possible to prevent Calciphylaxis. However, the following measures may be considered:

  • Undertaking appropriate treatment of underlying conditions such as kidney disease, diabetes, and autoimmune conditions, may help avoid Calciphylaxis
  • A healthy lifestyle can help prevent morbid obesity or severe malnutrition that are risk factors for the condition
  • Limiting the use of certain medications (including steroids and blood thinners)

What is the Prognosis of Calciphylaxis? (Outcomes/Resolutions)

  • The prognosis of Calciphylaxis is generally poor. The condition usually progresses and results in death, despite suitable treatment
  • However, the prognosis may also be dependent upon the severity of the primary dermatitis (skin disorder)

Additional and Relevant Useful Information for Calciphylaxis:

Calciphylaxis is also known as Calcific Uremic Arteriolopathy (CUA).

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team


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