Necrotizing vasculitis (NV) is a rare disease characterized by the formation of large numbers of blood clots within the body. These clots can block vital organs such as the heart or brain, causing death due to organ failure. NV usually develops after trauma or other severe injury to the skin and underlying tissues. Most cases occur in older adults with no known risk factors.
The most common site of NV is the legs, but it can affect any part of the body. The condition occurs when there are too many blood vessels supplying blood to the affected area. The arteries and veins that supply blood to these areas become clogged with a buildup of plaque or other debris. When this happens, they cannot carry enough oxygenated blood from one place to another properly.
A person with NV will have swelling and pain at the site of the clot. Sometimes there is bleeding into surrounding tissue. Other times, the clot forms inside a vein or artery and travels through the bloodstream to cause problems elsewhere in your body. A small number of people develop NV without any obvious source of infection or injury. However, those individuals are considered to be at high risk for developing complications if they do not receive prompt medical attention.
Prognosis is good for most people with necrotizing vasculitis. If treated early, many patients can recover completely. Some people, however, suffer long-term complications such as pain and numbness lasting for months to years.
There is no known way to prevent necrotizing vasculitis, but it is usually treated successfully with surgery to remove the damaged blood vessels and clots. In some situations, medications are used to help treat the condition or manage complications.
What causes necrotizing vasculitis?
Necrotizing vasculitis is a condition in which the small blood vessels that supply the skin and other tissues become damaged or destroyed. It often leads to the formation of blood clots, which can narrow or block the flow of blood through the vessels. When this happens within certain types of blood vessels, it can result in a range of serious complications involving your muscles, skin, organs, and other parts of your body.
What are the symptoms of necrotizing vasculitis?
The most common signs and symptoms of necrotizing vasculitis include:
Swelling, redness, and sometimes blistering of the skin in the affected area. It may look like a rash or a sunburn.
Muscle pain or weakness that usually begins in one part of your body and spreads to other parts. The pain may be severe.
Pain when you move the affected part of your body.
Feeling faint or dizzy
Loss of appetite
Chills and fever
Diarrhea or constipation
Nausea or vomiting
How is necrotizing vasculitis diagnosed?
Your doctor will perform a physical examination. In addition, they likely will order tests to confirm the presence of this condition. Necrotizing vasculitis can be difficult to diagnose because some of its symptoms are similar to those for other conditions. Your health care provider will take a detailed medical history and ask if you have been exposed to any risks that could have caused the condition, such as surgery or recent injections.
Tests that may be used to diagnose and assess necrotizing vasculitis include:
Blood tests to measure the number of platelets (cells that help blood to clot) in the blood
Blood tests to measure levels of certain chemicals, such as creatinine or potassium
Electrocardiogram (ECG) to check the electrical activity of the heart
Chest x-ray to look for signs of pneumonia or other chest problems
Heart ultrasound, which uses sound waves to make a video of the interior of the heart
Magnetic resonance angiogram (MRA), which uses a combination of large magnets and radio waves to produce detailed images of the blood vessels and the blood flowing through them
Computed tomography (CT or CAT) scan, which uses a combination of X-rays and computer technology to produce cross-sectional images of your body to identify areas of infection, injury, or blockage in the blood vessels
Treatment for necrotizing vasculitis
Specific treatment for necrotizing vasculitis will be based on the following:
Your age, overall health, and medical history
Extent of the disease
Your tolerance for specific medications, procedures, or therapies
Expectations for the course of the disease
Your opinion or preference
Treatments may include:
Antibiotics, to prevent infection
Treatments to remove blood clots that have formed in your blood vessels, such as a procedure called thromboembolectomy or the use of medications to break up clots
Surgery to repair damaged blood vessels
Long-term treatment for the symptoms and complications caused by the damage to the blood vessels, such as kidney failure or skin sores (called ischemia)
Treatment of any conditions or disease that may have predisposed you to necrotizing vasculitis, such as diabetes, systemic lupus erythematosus (lupus), leukemia, or certain medication use
Clot-busting drugs such as tissue plasminogen activator (tPA), which are used in ischemic limbs to restore blood flow to the affected area and relieve pain.
Prevention of necrotizing vasculitis
The risk factors for necrotizing vasculitis are not well understood. However, some people should be alert to the signs and symptoms of this disease and seek medical care immediately if they develop. This may prevent the condition from becoming life-threatening.
If you’ve been diagnosed with necrotizing vasculitis, your doctor may recommend that you carry a card or wear an identification bracelet stating that you have this condition. This will alert medical personnel to the need for added care if you suffer an injury or undergo surgery. Also, it may be helpful to wear medical alert jewelry.
Any additional information found on this disease
Can necrotizing vasculitis be prevented?
Some potential causes of necrotizing vasculitis are known. Necrotizing vasculitisthough the cause is not always clear, may be caused by infection with a type of bacteria (Group A streptococcus). This type of bacteria commonly cause strep throat, scarlet fever, and other skin infections. However, Group A streptococcus does not always cause necrotizing vasculitis.
Other causes include:
Autoimmune disorders, such as lupus
Blood clotting problems
Poison Ivy/Poison Oak exposure
Rheumatoid arthritis or other autoimmune diseases
Tissue or organ transplantation (usually within 6 months of transplant)
Pregnancy (after the first trimester)
Trauma (blunt or penetrating injury to tissue)
Vasculitis is a general medical term that describes inflammation of blood vessels. When a form of vasculitis destroys blood vessels, it is known as necrotizing, or “killing,” vasculitis. Necrotizing vasculitistype usually affects small blood vessels, such as in the skin, nerves, and internal organs.
The small blood vessels become weak and are prone to leaking blood and proteins, eventually becoming blocked by clots. This process deprives the surrounding soft tissue of nutrients and oxygen and causes ischemia (tissue death).
Necrotizing vasculitis most commonly affects the skin, nerves, muscles, kidneys, and digestive tract, but it can also affect other parts of the body.
Necrotizing vasculitis most often occurs in people between the ages of 15 and 50 and is three times more common in males than females.
Necrotizing vasculitis may be triggered by:
Infections such as hepatitis C or B, cytomegalovirus (CMV), HIV, or mycobacterium tuberculosis
Excessive or binge drinking
Pre-existing autoimmune disorders such as lupus erythematosus, scleroderma, or rheumatoid arthritis
Smoking or asbestos exposure (potentially)
Sources & references used in this article:
Cutaneous necrotizing vasculitis and related disorders. by JN Gilliam, JD Smiley – Annals of Allergy, 1976 – ncbi.nlm.nih.gov
Cyclophosphamide therapy of severe systemic necrotizing vasculitis by AS Fauci, P Katz, BF Haynes… – New England Journal of …, 1979 – Mass Medical Soc
Clinical and histopathologic spectrum of necrotizing vasculitis: report of findings in 101 cases by NP Sanchez, HM Van Hale, WPD Su – Archives of dermatology, 1985 – jamanetwork.com
Necrotizing vasculitis by WM Sams Jr – Journal of the American Academy of Dermatology, 1980 – Elsevier
The necrotizing vasculitides: a new pathogenetic classification by D Alarcón-Segovia – Medical Clinics of North America, 1977 – Elsevier
Cutaneous necrotizing venulitis by NA Soter, JL Diaz-Perez – Dermatology in general medicine, 1993 – researchgate.net
Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis by X Puéchal, G Said, P Hilliquin, J Coste… – … : Official Journal of …, 1995 – Wiley Online Library