Calciphylaxis is the accumulation of calcium in the blood vessels of the skin and fatty tissue. Calcium also builds up in the arterioles, the smallest parts of the arteries. The affected tissue is located just beneath the skin, around internal organs, in bone marrow, and in the breast, as well as within the muscular system. The calcium hardens, blocking vessels and arterioles, which slows or stops blood flow and often forms blood clots. Cells become deprived of oxygen they need to survive, which negatively affects the surrounding tissue. The spread of calciphylaxis is quick and painful.
The cause of calciphylaxis is unknown at this time. Originally, doctors thought the onset of kidney disease caused it. However, people with healthy renal systems also develop calciphylaxis. How the body breaks down calcium may be a factor, as might problems with blood clot formation or the body's ability to prevent calcium buildup. Also linked to calciphylaxis are abnormal vitamin and hormone levels.
The kidneys are responsible for how the body processes waste, including calcium. They also filter blood. Because of that, kidney disease is the main risk factor for calciphylaxis. Dialysis and kidney transplants increase this risk. Other diseases also increase the risk of this secondary disease, including inflammatory diseases like rheumatoid arthritis, hyperparathyroidism, and ulcerative colitis. Autoimmune disease, metastatic cancer, and diabetes can also increase the likelihood of developing calciphylaxis.
Females develop calciphylaxis at a much higher rate than males. According to the New England Journal of Medicine, obesity increases the risk of developing calciphylaxis four-fold. Taking certain medications, and imbalances in hormonal, protein and vitamin levels, can have similar results. Exposure to ultraviolet light or aluminum is another risk factor, as are skin trauma and rapid weight loss.
Calciphylaxis limits the amount of blood flow and oxygen in the cells. When that happens, lesions develop on the skin. They form a net-like pattern at first, then grow larger and become dark bluish-purple or black wounds. This happens because of skin necrosis -- the tissue is dying. The wounds do not heal, but become larger and eventually cause serious infections.
There are no specific tests for diagnosing calciphylaxis. The presence of skin necrosis is usually enough to make the diagnosis in patients with kidney disease. However, in patients with healthy renal systems, the doctor must examine the medical history and lab results and perform a tissue biopsy.
A biopsy cannot is not always possible because biopsies cause skin wounds. If the skin is already necrotic, this is one more wound that will not heal properly. Biopsies are also not performed on sensitive areas or infected skin, which can severely limit their usefulness in cases of calciphylaxis. Doctors turn to imaging if a biopsy is not possible or inconclusive. They look for net-like patterns under the skin.
Calciphylaxis causes a lot of pain. Therefore, pain management is a top priority of treatment. Another is slowing calcification with medication, which can also help prevent unwanted blood clots. Dialysis and oxygen therapy are additional options. The latter increases the amount of oxygen in cells. Surgery to remove an overactive thyroid gland may also offer relief.
Wound care is a major part of calciphylaxis treatment. Infection is common and prevention is vital. To do so, doctors will remove damaged tissue whenever possible, a procedure called debridement. Unfortunately, debridement causes a lot of pain, which can make it an unviable solution. In such cases, the doctor may apply wet bandages and administer antibiotics.
A positive prognosis for calciphylaxis largely depends on early diagnosis. Having a healthy renal system is also helpful. Unfortunately, the prognosis is less positive for people with end-stage renal disease. Either way, people with calciphylaxis can improve their outlook by following the doctor's prescribed disease management program and continuing to manage any co-occurring medical conditions.
Experimental treatments for calciphylaxis are promising. Researchers are currently testing gene therapy targeting certain molecules. The use of sodium thiosulfate, a compound that slows calcification, is also being explored, and some vitamin supplements and blood clotting medications show promise. It is evident that the medical world takes calciphylaxis very seriously, and trials will continue until researchers discover an effective treatment program.
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