What is Calciphylaxis?
Calciphylaxis is a pathological condition in which there is abnormal calcium deposition in the body, which primarily affects the small blood vessels and causes extensive skin damage. This calcium deposition makes the blood vessels hard and hence Calciphylaxis is also called as vascular calcification. Calciphylaxis affects the blood vessels that supply blood to the subcutaneous fat and the skin. Calcium deposition can sometimes occur in the blood vessels of certain vital organs of the body, which may cause certain life threatening complications even though it is the skin that is primarily affected by Calciphylaxis.
This is a rare condition and is found mostly in people with end stage kidney disease, as in such cases the body is unable to maintain normal levels of various minerals including calcium. Calciphylaxis may also be at times related to aluminium toxicity and vitamin D supplementation. If Calciphylaxis affects internal organs of the body, then it can be catastrophic and in about 80% of the cases the patient is not able to survive due to Calciphylaxis.
What Causes Calciphylaxis?
As stated above, Calciphylaxis is caused due to abnormal calcium deposits in the blood vessels which supply blood to the subcutaneous fat and the skin. This condition is mostly found in people with kidney diseases who have abnormal calcium levels, but there are some cases of Calciphylaxis in people who have normal calcium levels and do not have any kidney dysfunction.
Calciphylaxis Caused Due to Kidney Failure: This is the primary cause of Calciphylaxis. One of the functions of the kidney is to regulate and control the levels of different minerals in the body which includes calcium. If an individual has a dysfunctional kidney, then the kidney is not able to regulate or control the levels of these minerals in the body including calcium and there is an increase in the levels of calcium in the body. These calcium deposits gradually start to accumulate on the blood vessels, which then slowly become calcified. This calcification over time leads to Calciphylaxis.
Some of the Other Less Common Causes of Calciphylaxis are:
- Aluminum toxicity.
- Blood clotting disorders.
- Medications affecting calcium levels in the body.
- Inflammatory conditions and use of steroids which are widely used to treat such conditions.
- Vitamin D supplementation.
What are the Symptoms of Calciphylaxis?
The main presenting feature or symptom of Calciphylaxis is the development of skin discoloration leading to gangrene and ulcers. The lesions caused by Calciphylaxis are basically seen in the abdomen, thighs, buttocks, lower part of the legs and feet with itching, pain, and burning sensation in the affected areas. During the early stages of the disease when there is development of the lesions, there will be mottling of the skin and the skin will have a purplish discoloration.
There will also be bleeding with blood filled blisters. Once the disease condition progresses, the development of the lesions progress and they take a star shaped appearance with a black center. These lesions become very firm and are tender to palpation. In the advanced stages of Calciphylaxis, there is a formation of gangrene and multiple deep ulcers on the affected area.
How is Calciphylaxis Diagnosed?
The lesions formed from Calciphylaxis are quite similar to those formed by other vascular conditions and hence it is vital to rule out other vascular conditions causing the symptoms. Once other conditions have been ruled out, then the symptoms may point towards Calciphylaxis and investigations for that is started. Some of the tests done for confirming the diagnosis of Calciphylaxis are:
Blood tests to check for the levels of calcium in the body along with the levels of phosphorus, creatinine and PTH levels. A plain x-ray of the affected area may be taken to look at the extent to which there is damage done to the skin. A skin biopsy may also be done for confirmation of the diagnosis of Calciphylaxis.
How is Calciphylaxis Treated?
The treatment for Calciphylaxis depends on the severity of the condition and the extent to which the blood vessels are calcified. If the disease has progressed to gangrene and ulcers, then the patient will be sent to wound care specialist to prevent the infection from spreading to the point of septicemia which is a life threatening condition. The patient will be treated with antibiotics and any necrotic tissue will be debrided. Another priority for the physicians is to restore normal blood and oxygen supply to the areas where tissue necrosis has not taken place because of lack of blood and oxygen.
Hyperbaric oxygen therapy is one such way to prevent any tissue affected due to Calciphylaxis. For clotting issues, clot dissolving medications will be given so as to restore blood flow to the affected area. Apart from this, for treating Calciphylaxis as a disease the focus is on reducing the calcium and phosphate levels. This may be done by way of dietary planning with foods that are low on calcium and phosphate. There are certain medications which are designed to reduce calcium levels in the body. These medications are:
- Calcimimetics. These are medications which act like insulin resulting in the parathyroid gland to cut down the production of parathyroid hormone, which in turn reduces calcium levels in the body.
- Bisphosphonates. These are medications which prevent breakdown of bones and thus reducing calcium levels in the body.
- In case of end stage renal disease causing Calciphylaxis, regular dialysis is done to remove any excess calcium and phosphate in the body.
It is important that Calciphylaxis be treated in a timely manner so as to reduce the chances of complications. Some of the complications of Calciphylaxis are:
- The gangrene getting severe enough that it becomes necessary to amputate the particular area or limb.
- Development of septicemia because of spread of infection to the blood, which has an extremely poor prognosis.
- Hypocalcemia as a result of calcium lowering medications.
- Myocardial infarction, gastrointestinal bleeding, and organ failure in case the internal organs are affected by Calciphylaxis.
- UpToDate. (2021). Calciphylaxis (calcific uremic arteriolopathy). https://www.uptodate.com/contents/calciphylaxis-calcific-uremic-arteriolopathy
- American Journal of Nephrology. (2018). Calciphylaxis: Risk factors, diagnosis, and treatment. https://www.karger.com/Article/FullText/492142
- Journal of Cutaneous Medicine and Surgery. (2016). Calciphylaxis: Diagnosis, Pathogenesis, and Treatment. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4841624/