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Calciphylaxis occurs when calcium deposits build up in the blood vessels, obstructing blood flow to certain skin regions. This disease results in the formation of blood clots, the development of painful skin ulcers, and the potential for severe infections that can be fatal.
In rare instances, calcium deposits may form internally in various organs like the eyes, lungs, brain, muscles, and intestines. When it affects the skin’s surface, calciphylaxis is very painful, causing open wounds similar to bedsores. The skin and surrounding tissue near the wound undergo necrosis, causing the damage from the dead tissue to extend outward. The healing process for these wounds is slow and may lead to significant complications.
Calciphylaxis is typically experienced by individuals who has kidney failure, is undergoing dialysis or has undergone a kidney transplant. This condition can also manifest in individuals without pre-existing kidney disease.
Symptoms
Calciphylaxis typically presents with intense pain, often initiating prior to the manifestation of lesions or visible symptoms. The affected areas can exhibit heightened sensitivity to pressure or touch, further intensifying the overall pain.
Other symptoms include:
- Painful, deeply seated lumps that eventually form open sores covered in dark-brown crusts, persistently resisting healing. These lesions typically manifest in fatty skin areas,such as the abdomen and thigh, although they can develop in any part of the body.
- Skin with large purple net-like patterns.
- Infections resulting from non-healing wounds.
- Vision problems, internal bleeding, and muscle damage.
Causes
Although the precise cause of calciphylaxis is yet unknown, the information that is currently available points to multiple potential causes.
Recent research indicates that individuals with this condition often exhibit abnormalities in blood-clotting factors, which are substances in the blood that aid in preventing excessive bleeding. These irregularities may result in an increased frequency of the formation of small blood clots compared to the usual occurrence.
Other studies suggest that imbalance in the metabolism of calcium can result in calciphylaxis. This results in the deposition of calcium in the arterioles which ultimately causes blood clots to form there. Skin and adipose tissues can become oxygen- and nutrition-deprived due to blood clots.
Risk factors
Calciphylaxis primarily affects individuals in the advanced stages of kidney failure, with potential risk factors including being female, being obese, diabetes, abnormalities in blood-clotting factors, long-term dialysis, and, at times, kidney transplantation.
Other risk factors include:
- An imbalance in the body’s levels of phosphorus, calcium, and aluminum
- Hyperparathyroidism, a disorder marked by an excess of parathyroid hormone (PTH), which controls the body’s levels of calcium and phosphorus
- Uremia, or a condition in which the body accumulates substances like calcium and phosphorus that are typically excreted in the urine
- Certain drugs, like corticosteroids, calcium-binding agents, or warfarin
Diagnosis
Diagnosing calciphylaxis typically involves a comprehensive evaluation of symptoms, a thorough examination of one’s medical history, and a physical assessment to detect any changes in the skin or underlying tissue.
These tests may be ordered to help with the diagnosis:
- Lab tests: Blood and urine tests play a crucial role in diagnosing and assessing the severity of calciphylaxis. Blood samples analyze an array of compounds present in your bloodstream, including but not limited to calcium, phosphorus, parathyroid hormone, abnormalities in blood-clotting components, aluminum levels, urea nitrogen, creatinine, and albumin, among others. These measurements aid your healthcare provider in evaluating the functioning of your kidneys and liver.
- Imaging test: Vascular calcifications, or branch-like calcium deposits in the blood vessels, are frequently observed in severe kidney diseases and calciphylaxis can be seen on X-rays.
- Skin biopsy: A skin sample is usually obtained from the perimeter of a lesion or wound, typically around 4 or 5 millimeters deep. Analysis of the sample involves microscopic examination and the application of specific substances to induce color or chemical alterations, aiding in the definitive confirmation of the diagnosis.
Treatment
Currently, there is no known cure for calciphylaxis, but effective treatment approaches can help induce a state of remission. Recommended treatment options may encompass:
- Intensive wound care: The primary focus is on preventing infection and facilitating wound healing. Medication may be prescribed to manage pain associated with calciphylaxis or during the process of wound care. Antibiotics are utilized as part of the treatment to prevent and address wound infections.
To promote the healing of sores, surgical removal or debridement of damaged tissue affected by calciphylaxis may be necessary. Alternatively, other methods like wet dressings could be employed for tissue removal. - Increasing blood flow and oxygen to the skin: Hyperbaric oxygen therapy entails placing the individual in a sealed chamber with 100% oxygen at a slightly higher pressure than usual, compared to the normal 20% oxygen concentration. This method is highly effective in promoting the healing of wounds.
Administering low-dose tissue plasminogen activator (TPA) may help disintegrate blood clots in the skin’s tiny blood vessels. In order to restore blood flow to injured tissues, anticoagulant medicine, such as apixaban, may be prescribed to help prevent blood clots. - Reducing calcium deposits
- Taking medications: Cinacalcet, a drug that helps regulate parathyroid hormone (PTH), may be suggested. Sodium thiosulfate, a medicine that helps reduce the accumulation of calcium in the arterioles, may be administered thrice a week, typically during dialysis. The calcium and phosphorus balance can also be improved by taking additional drugs.
- Modifying medications: The dosage of any calcium or vitamin D supplements may be adjusted if it can potentially cause calciphylaxis. To eliminate any substances that could trigger calciphylaxis, such as iron, corticosteroids, or warfarin, the healthcare provider will review all medications one is taking.
- Dialysis: In the case of individuals undergoing kidney dialysis, adjustments to the dialysis prescription, including modifications to the medications utilized and the frequency of the dialysis sessions may be required.
- Surgery: Surgical excision of all or a portion of the parathyroid glands may be advised if an excessive production of parathyroid hormone or hyperparathyroidism leads to aberrant calcium metabolism.
