What causes Urticarial Vasculitis?

Urticarial vasculitis is a form of vasculitis that affects the skin, causing wheals or hives and/or red patches due to swelling of the small blood vessels. It has two forms: One with normal levels of proteins called complements; the other with low levels of complements; it’s called hypocomplementemic vasculitis.

The cause of most cases of urticarial vasculitis is unknown. It may be associated with a number of diseases, especially systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. Some cancers, including leukemias, colon and pancreatic, and infections like Hepatitis B and C can cause this form of vasculitis. So can some drugs, including antibiotics, ACE inhibitors used for treating high blood pressure, and certain diuretics.

How common is it?

Urticarial vasculitis is uncommon. There are no well researched estimates of how frequent it is.

Who gets Urticarial Vasculitis?

Both males and females get the disease.

Symptoms

The most common symptoms are hives that cause itching, pain and a burning feeling. Skin patches often are red-rimmed with white centers, and unlike common hives may have petechia, or bleeding under the skin. The patches can be present for days and result in skin discoloration as they heal. Some patients may also have fevers, joint and abdominal pain, shortness of breath and swollen lymph glands. Sometimes urticarial vasculitis even causes injury to vital organs including the gut, lungs and kidneys.

Diagnosis

Diagnosis is based on characteristic patches in the skin. Sometimes a biopsy is ordered to show inflammation in the skin and damage of small blood vessels with white blood cells. Since it’s often associated with a number of different diseases, it’s often necessary to do other tests and exams to rule out underlying conditions like lupus erythematosus or cancer. Tests of vital organs may also be indicated, especially when the blood levels of complement are low.

Treatment

Treatment depends on the extent of symptoms and organ involvement. When levels of complement are normal and there is no internal organ involvement or underlying disease, the symptoms may improve on their own or with minimal treatment. In this case, antihistamines or nonsteroidal drugs such as ibuprofen or naproxen may be helpful. For more severe cases, other drugs which affect the immune system may be needed, such as corticosteroids (prednisone, others), hydroxychloroquine, colchicine, dapsone; and chemotherapies like azathioprine or cyclophosphamide. Treatment may be intermittent, although it is not uncommon for patients to need treatment for several years.

What are the complications?

The most common serious complications are skin pigmentation and, occasionally, skin ulcers, plus damage to organs such as the lungs, eyes and kidneys.

Prognosis

The natural history of urticarial vasculitis depends in part upon the blood complement levels. In cases where these are normal, the prognosis is generally good. In cases where the complements are low, the disease may be more severe. When urticarial vasculitis is related to a disease such as lupus or cancer, its prognosis is often governed by the prognosis of the underlying disease

What’s new in Urticarial Vasculitis?

Progress is being made in understanding how urticarial vasculitis comes about and how to better treat it.

Revision:  September 2012

The Vasculitis Foundation gratefully acknowledges Dr. Eric L. Matteson from the Mayo Clinic, Rochester, MN, for his expertise and contribution in compiling this information.