What is Leukocytoclastic Vasculitis?
Vasculitis is a condition that causes inflammation of the blood vessels, which can result in tissue damage and possibly harm to organs. Vasculitis is classified based on the size of the vessels it affects, so it can be small, medium or large vessel vasculitis and can occur on its own or be connected to other medical conditions or diseases.
Examples of small vessel vasculitis include (ANCA) associated vasculitis, Behçet’s disease, and Cogan’s syndrome. This type of vasculitis can also occur due to immune system reactions in conditions like rheumatoid arthritis, lupus, Henoch-Schönlein purpura, and certain other specific conditions. Additionally, different factors such as certain medications, underlying cancers, and infections (like hepatitis B, hepatitis C, and syphilis) can cause small vessel vasculitis or leukocytoclastic vasculitis.
Leukocytoclastic vasculitis, which specifically affects small blood vessels in the skin, is characterized by inflammation caused by immune system reactions. Mostly, this condition affects the skin causing symptoms like palpable spots under skin, and primarily occurs on lower extremities. Other symptoms related to the involvement of small skin vessels may also be present. In less than 30% of the cases symptoms outside the skin might develop.
If doctors suspect leukocytoclastic vasculitis, they will likely perform a punch biopsy with additional diagnostic tests. If there are no systemic symptoms, blood tests including a full blood count, liver function tests, basic metabolic panel, and a urine test will be done. However, if there are signs of the condition affecting the whole body, a more extensive evaluation may be required.
Typically, most cases of vasculitis affecting the skin clear up on their own, with 90% of the cases resolving in weeks to months from their start. However, if the condition persists, treatment will be based on how severe the disease is and can include oral steroids or other medications that can help manage symptoms and prevent further organ damage.
What Causes Leukocytoclastic Vasculitis?
Leukocytoclastic vasculitis is a condition where blood vessels become inflamed. Its specific cause often remains unknown in up to half of all cases. However, infections or certain medications frequently trigger this type of vasculitis. It can also be associated with diseases where the immune system attacks the body, chronic infections, and various types of cancer.
One specific example is when it follows a streptococcal infection of the upper respiratory tract. Various other infections can also lead to this condition. These include diseases caused by bacteria like Mycobacterium and Staphylococcus aureus, as well as viruses like HIV. It’s also possible to see this condition in people with ongoing infections with hepatitis B, hepatitis C, or syphilis.
Numerous medications can lead to this condition. Typically, symptoms appear 1 to 3 weeks after starting a new drug. Some of these medications include types of antibiotics, furosemide (a medicine that helps your body get rid of extra salt and water), allopurinol (used to prevent gout), NSAIDs (medications often used to relieve pain), amiodarone (a heart medication), and many others.
Cancers, specifically blood cancers like lymphomas and leukemias, and visceral tumors such as intestinal and lung cancers, could also trigger this condition.
Lastly, it can be associated with systemic diseases. These diseases could involve connective tissue disorders (like systemic lupus erythematosus, where the immune system mistakenly attacks healthy tissue, and Sjogren syndrome), inflammatory bowel disease, Behcet disease (a rare disorder that causes blood vessel inflammation), rheumatoid arthritis, and others. These can all potentially be associated with leukocytoclastic vasculitis.
Risk Factors and Frequency for Leukocytoclastic Vasculitis
Leukocytoclastic vasculitis, a condition confirmed through a biopsy, affects roughly 45 out of a million people every year. This condition can occur at any age and in both genders, but it’s usually seen in adults. However, a specific type of this condition, known as HSP, typically affects children and young adults. Up to 270 cases of HSP occur per million children per year. Here, too, boys seem to be affected slightly more than girls.
- The yearly rate of leukocytoclastic vasculitis is about 45 cases per million people.
- This condition seems in all ages and in both males and females, but usually it is seen in adults.
- A special type of this condition called HSP is more common in children and young adults.
- Every year, up to 270 cases of HSP are seen per million children.
- HSP is slightly more common in males.
Signs and Symptoms of Leukocytoclastic Vasculitis
Leukocytoclastic vasculitis is a condition that often shows up on the skin. It usually appears 1 to 3 weeks after an event that triggers it. Signs of this condition may include red patches of skin and purplish areas that you can feel, usually on both sides of lower parts of the body like the legs and buttocks. It is uncommon for these signs to show up on one side of the body only or in specific localized areas. Other signs can include blood-filled blisters, pus-filled patches, lumps, areas of crusted-over ulcers, or a lace-like network of purple discolorations on the skin. These skin changes can vary in size from very small (1 mm) to 1 cm in diameter. These skin changes can appear all at once or in several batches, each batch producing lesions at different stages of development.
Another feature of this condition is called Koebnerization, which is when skin changes appear in areas where the skin has been injured. However, this is rare. In fact, the opposite has been observed with this condition: it has been noted to disappear when pressure bandages are applied after a biopsy. These skin changes generally don’t cause any symptoms, but they may cause itching, burning or stinging.
Leukocytoclastic vasculitis can also show up in other parts of the body, but this is less common. When it does, it can cause low-grade fevers, discomfort, weight loss, muscle pain, and joint pain. About 30% patients may experience these symptoms. Less frequently, it may affect the kidneys, digestive system, lungs, or nervous system. In these cases, it may actually be a different kind of vasculitis, called microscopic polyangiitis or polyarteritis.
Other symptoms can give clues about what is causing the vasculitis. For example, in children, leukocytoclastic vasculitis combined with inflammatory arthritis, stomach pain, swelling and blood in the urine can point to a condition called Henoch-Schoenlein purpura. Vasculitis is less likely to be the first sign in people with rheumatoid arthritis, Sjogren’s syndrome or lupus, as these patients are usually diagnosed for several years before they develop vasculitis. However, in lupus, vasculitis can sometimes be the first sign. In these cases, the patients will likely have other symptoms of lupus as well.
Testing for Leukocytoclastic Vasculitis
When a doctor suspects a patient is suffering from leukocytoclastic vasculitis, a skin condition where blood vessels in the skin become inflamed, a skin biopsy with a technique called direct immunofluorescence is the most reliable method for diagnosis. This involves taking a small sample of the patient’s skin and examining it under a microscope to look for signs of the disease.
Once this diagnosis is made, the doctor will usually order further tests to figure out the underlying cause of the disease. These tests can vary, based on what the doctor believes could be causing the problem. Basic tests usually include complete blood counts (which measure different types of cells in your blood), liver and kidney function tests, and a urinalysis (a test that checks for different substances in the urine).
If there are concerns that the disease might be affecting other parts of the body, the doctor might order more extensive tests. These can include tests for viral hepatitis and HIV, tests for antibodies that are often present in conditions like systemic lupus erythematosus and Sjogren syndrome, rheumatoid factor (a protein that’s often present in people with rheumatoid arthritis), and tests for other substances in the blood that can indicate inflammation.
The type of testing done will depend on each individual patient’s case, and is decided based on the doctor’s expertise and the patient’s symptoms and medical history.
Treatment Options for Leukocytoclastic Vasculitis
Most cases of a skin condition called idiopathic cutaneous leukocytoclastic vasculitis, which causes inflammation in tiny blood vessels, are generally mild and can get better with a few simple steps. This may include raising the leg, resting, wearing compression stockings (tight stockings that gently squeeze your leg), and taking antihistamines (medications typically used to treat allergies).
In cases where the condition persists or becomes chronic, corticosteroids, a type of drug that reduces inflammation in the body, are used over a period of 4-6 weeks. For rare cases that don’t respond to regular treatments, stronger treatments may be used. These are specific medications that weaken the immune system, including methotrexate, azathioprine, mycophenolate mofetil, dapsone, cyclophosphamide, and a type of treatment called intravenous immunoglobulin.
If a certain medication is found to be causing the condition, it is really important that you stop taking that medication to help get better. In instances where an infection is causing the vasculitis, treating the infection typically results in the condition getting better.
If the vasculitis is linked to cancer, treating the cancer often helps improve the condition. If the vasculitis is associated with an underlying condition that affects the connective tissue or an autoimmune disease, such as systemic lupus erythematosus (a disease where the immune system attacks its own tissues) or rheumatoid arthritis (a long-term condition causing pain, swelling, and stiffness in the joints), controlling the underlying disease more effectively might be required to treat the vasculitis and prevent its return.
Mild cases of HSP, another type of vasculitis that often shows up as a skin rash, usually do not require heavy medications, and the skin rash and joint pains usually respond to nonsteroidal anti-inflammatory drugs (also known as NSAIDs, which reduce inflammation and pain). Though, in severe instances where the kidneys or other systems in the body are significantly impacted, high-dose corticosteroids and/or other stronger treatments may be needed.
What else can Leukocytoclastic Vasculitis be?
When diagnosing certain conditions, doctors will need to differentiate between several possibilities. These may include:
- Thrombocytopenic purpura
- Benign pigmented purpura
- Schamberg disease, which is an unexplained condition where red blood cells leak into the skin of the lower legs due to weak or leaky blood vessels, slow blood flow, or exercise. Frequent presence of darker skin patches on the lower legs is a common sign of this condition.
Furthermore, a skin biopsy can help distinguish benign pigmented purpura from leukocytoclastic vasculitis. This distinction is made by the absence of vasculitis characteristics like unnecessary natural cell death and the disruption of the blood vessel wall by the inflammatory cells.
What to expect with Leukocytoclastic Vasculitis
Leukocytoclastic vasculitis, a type of inflammation of small blood vessels, usually has a low mortality rate of about 2%. This rate is primarily dependent on whether the disease affects your whole system or just your skin. In about 90% of cases, patients see the skin lesions disappear on their own within a few weeks to months. However, the remaining 10% might deal with the disease for an average of 2 to 4 years.
It is also worth noting that if you’re experiencing joint pains without fever, this could indicate a long-term (chronic) progression of the disease.
Possible Complications When Diagnosed with Leukocytoclastic Vasculitis
Uncommon complications of cutaneous leukocytoclastic vasculitis, a disorder causing inflammation of blood vessels in the skin, include skin sores that may become infected if not properly cared for. Wound care plays a vital role in managing these skin sores, however, they typically don’t heal until the underlying condition is treated with immune system suppressing therapies such as corticosteroids.
- Cutaneous leukocytoclastic vasculitis causing inflamed blood vessels in the skin
- Uncommon skin sores which may become infected without proper care
- Importance of wound care in managing skin sores
- Typical healing of skin sores only once underlying condition treated
- Treating with immune system suppressing therapies such as corticosteroids
Preventing Leukocytoclastic Vasculitis
Recognizing signs early, particularly with drug-induced leukocytoclastic vasculitis (a type of inflammation of blood vessels often triggered by medications), is very important. Patients should be informed about the potential risks linked with the medication before starting it. Those with idiopathic cutaneous leukocytoclastic vasculitis, a version of this condition that happens without a known reason, usually have a positive outcome.
However, in cases where another underlying condition caused the vasculitis, closer and continuous monitoring will be needed. Patients should be informed how crucial it is to take their medication as instructed and attend regular check-ups. They should also be made aware that it may take several weeks to months for this blood vessel inflammation condition to improve.
