What Is Calciphylaxis?
Calciphylaxis is a skin disorder characterized by skin lesions that have areas of necrosis and are ischemic in nature. Calciphylaxis, also known as non-uremic calciphylaxis, is a disease that is caused by the calcification of the medial layer of small arteries. This calcification causes endothelial injury and the formation of microthrombi, resulting in narrowing of the arteries. In severe cases, the condition can lead to tissue ischemia and necrosis, or even ulceration.
Calciphylaxis Symptoms
A physical examination is a very important part of diagnosing calciphylaxis. It helps to visualize the lesions and determine the extent of skin integrity. The condition can begin with a stinging pain in the affected area and may progress to blisters and hardened skin. Patients often mistake the initial redness for a bruise.
In the later stages of calciphylaxis, an open wound may develop. Those wounds are usually slow to heal and are prone to major complications. Because the skin is the body’s first line of defense, an overreaction of the immune system can lead to the development of sepsis, a potentially fatal infection.
A point-of-care ultrasound may be a useful diagnostic tool for calciphylaxis. The presence of thin, linear hyperechoic bands parallel to the epidermal surface and blood vessel walls may help determine the diagnosis. Plain radiographs have also been used to diagnose the condition. A point-of-care ultrasound may also be useful for evaluating breast tissue, particularly during breast biopsies.
Calciphylaxis Causes
The causes of calciphylaxis are not yet fully understood, but the risk factors for developing the condition include chronic kidney disease, autoimmune diseases, and trauma such as a transplant. Exposure to substances that act as sensitizers and triggers may also increase the risk of calciphylaxis. This disease typically affects patients in the final stages of kidney failure, who are either on dialysis or are in need of kidney replacement surgery.
Clinical manifestations of calciphylaxis can include painful ulcers on the thighs and abdominal regions. These lesions are often heterogeneous in appearance. Patients often present with multiple tender violaceous nodules or plaques that progress to necrosis. The patient may also experience surrounding pruritus.
Causes of calciphylaxia are varied and can include a number of factors, including exposure to ultraviolet light, aluminum, and rapid weight loss. However, the symptoms of calciphylaxis are similar to those of other clinical conditions, and it’s important to rule out other conditions to ensure that you are not suffering from calciphylaxis.
How Is Calciphylaxis Diagnosed?
The first step is to diagnose your symptoms. This may involve a skin biopsy, a bone scan, or a kidney function test. If you notice changes in your skin, you may have calciphylaxis. It is also important to pay attention to the way your skin feels and looks. Swelling and redness of the skin are other signs of calciphylaxis.
Skin biopsy
Diagnosis of calciphylaxis may be difficult due to its ill-defined clinical presentation and the difficulty of treating it. The most reliable method of diagnosis is a skin biopsy and histological examination, although medical imaging is also useful in some cases. Treatment must target the underlying etiology of pain in order to improve patient health.
The most common form of calciphylaxis is sepsis secondary to infected wounds. While this is the most common form of the condition, it is important to rule out other causes if possible. A patient should seek prompt medical attention if symptoms persist or worsen.
The most reliable way to diagnose calciphylaxis is a skin biopsy, which is typically taken 4 to 5 millimeters deep at the edge of a lesion. The tissue is then examined under a microscope to determine whether it contains calcifications. A diagnosis of calciphylaxis will be confirmed if the lesion shows a signature of net-like calcifications in the skin and subcutaneous tissue.
Bone scan
Using a bone scan for calciphylaxis is a noninvasive method to diagnose the disorder. A bone scan is fairly sensitive and can be used in place of biopsy. In one study, bone scans revealed abnormalities in 34 of 36 patients. The most common abnormality was an increase in bone uptake in the calves and ulceration.
A bone scan can also detect calciphylaxis by revealing microcalcifications in soft tissue. However, this invasive procedure is time-consuming and carries risk of infection and poor wound healing. It is recommended for those who have a suspicion for this disease.
This method has high sensitivity and specificity in diagnosing calciphylaxis. The uptake of bone-scan images by soft tissues is high and is useful for early diagnosis.
Kidney function tests
People with calciphylaxis may have a family history of kidney problems or have recently undergone a kidney transplant. The condition can also be the result of a calcium imbalance. The hormone PTH is responsible for maintaining calcium levels in the body. Certain autoimmune diseases can change the balance, which can cause calciphylaxis.
The most common symptom of calciphylaxis is skin ulceration and necrosis, accompanied by significant pain. It is thought that the condition is caused by the accumulation of calcium in the medial wall of small blood vessels. Medial calcification of these blood vessels can also cause thrombotic occlusion and cause significant pain. The disease is rare but can be fatal if not diagnosed early.
Patients with calciphylaxis should undergo a series of tests including bone scintigraphy and blood tests. The use of a CT scan, ultrasound and MRI can also be helpful in diagnosing this condition.
What is the Treatment for Calciphylaxis?
In cases where other tests have been inconclusive, diagnostic imaging tests may be performed. While there is no specific cure for calciphylaxis, it can be controlled and even go into remission if the right treatment is administered. Hyperbaric oxygen therapy, which involves placing the patient under a 100% oxygen atmosphere, is one such treatment. It has been shown to promote wound healing.
IL sodium thiosulfate
Sodium thiosulfate is an intravenous medication used in the treatment of calciphylaxis. In addition to its role in preventing the decalcification of the uremic vessels, STS has antioxidant properties that reduce the risk of thrombosis and inflammation. It has also been shown to reduce the formation of hydroxyapatite.
This treatment is low-risk and highly targeted. Unlike topical preparations, sodium thiosulfate is injected directly into the calf muscle. This allows for better penetration through the skin, which avoids the risk of systemic distribution. In addition, intralesional sodium thiosulfate is already used safely to treat extravasation injury. Moreover, it may be effective in treating localized calciphylaxis and may be of great value for patients who do not need dialysis.
IL sodium thiosulfates was given to a patient who had chronically worsening ulcers on her left leg. In this case, the treatment was successful in treating the ulcer. A 1-mL intravenous injection of sodium thiosulfate was given around the rim and in the center of the ulcer. After treatment, the patient’s ulcers healed within six to eleven months.
Hemodialysis
Hemodialysis is a necessary treatment for patients suffering from calciphylaxis. The condition is often debilitating and may require a parathyroidectomy and other medical procedures. Additionally, patients may need support with nutrition, psychological support, and pain management. The condition can be fatal. It can cause kidney failure and may lead to infections such as sepsis.
There are a number of risk factors for calciphylaxis, including diabetes mellitus and CKD. Patients who are female, obese, and of white race are also at increased risk. Hyperphosphatemia and hypercalcemia are also risk factors. People who are on warfarin therapy may also develop calciphylaxis.
The initial study was based on data from 89 patients registered in the UKCS since 2012. The data were used to compare patient characteristics, management strategies, and outcomes at follow-up until May 2020. A propensity-matched group of CRISIS-HD patients was also included in the analysis.
