Urticarial Vasculitis Web 72012
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www.VasculitisFoundation.org 800.277.9474 How common is Urcarial vasculis disease? Urcarial vasculis is uncommon. There are no well researched esmates of how frequent it is. Who gets Urcarial vasculis? Both males and females may be affected. Urcarial vasculis may be associated with a number of diseases. Hypocomplementemic urcarial vasculis especially is associated with rheumatologic diseases such as systemic lupus erythematosus, rheumatoid arthris and Sjögren’s syndrome. Some cancers including leukemias and solid cancers such as colon cancer, pancreas cancer and others also may cause urcarial vasculis. In addion, certain infecons like hepas B and C can cause this form of vasculis, as can some drugs, including anbiocs, ACE inhibitors used for treang high blood pressure, and certain diurecs. Sll, the cause of most cases of urcarial vasculis is unknown. Symptoms The most common symptoms are hives in the skin, which can cause itching, pain and a feeling of burning of the skin. The skin patches oſten are red rimed with white centers, and unlike common hives, may have petechia, or bleeding under the skin. The patches can be present for days, and can leave behind skin discoloraon as they heal. Some paents may also have systemic symptoms, meaning symptoms other than in the skin. Urcarial vasculis may cause fevers, joint and abdominal pain, shortness of breath, swollen lymph glands, and somemes even causes injury to vital organs including the gut, lungs and kidneys. Diagnosis Urcarial vasculis is diagnosed on the basis of characterisc patches in the skin. Biopsy of the skin may be needed to be sure of the diagnosis. The results of the biopsy can show inflammaon in the skin and damage of small blood vessels with white blood cells. Because urcarial vasculis can be associated with a number of different diseases, it is oſten necessary to perform examinaons to determine if an underlying disease such as lupus erythematosus or cancer is present. Because this form of vasculis can affect vital organs such as the kidneys, other tests, for example of kidney funcon and chest radiographs may be needed, especially when the blood levels of complement are low. Treatment egardless of the cause, urcarial vasculis is a treatable condion. The 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 such cases, anhistamines 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 corcosteroids (Prednisone, others), hydroxychloroquine, colchicine, daps one, and chemotherapies like azathioprine or cyclophosphamide. Treatment may be intermient, although it is not uncommon that paents need treatment for several years. What are the potenal complicaons of Urcarial vasculis? The most common serious complicaons are skin pigmentaon, and occasionally skin ulcers and damage to organs such as the lungs, eyes and kidneys. What is the prognosisof Urcarial vasculis? The natural history of urcarial vasculis 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 the urcarial vasculis is due to a disease such as lupus or cancer, the prognosis is oſten governed by the prognosis of the underlying disease. What is new in Urcarial vasculis? Progress is being made in understanding how urcarial vasculis comes about, and how to beer treat it. The Vasculis Foundaon gratefully acknowledges Dr. Eric L. Maeson from the Mayo Clinic, Rochester, MN, for his experse and contribuon in compiling this informaon. This brochure was made possible by an educaonal grant from Genentech, Inc., and Biogen Idec. Urcarial Vasculis Urcarial vasculis is a form of vasculis that affects the skin, causing wheals or hives and/or red patches. Unlike common hives, these erupons are actually due to vasculis of small blood vessels. There are two types of urcarial vasculis. One has normal levels of proteins called complements, and the other can have low levels of these complement proteins (so-called hypocomplementemic vasculis).
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Transcript of Urticarial Vasculitis Web 72012
www.VasculitisFoundation.org 800.277.9474
How common is Urticarial vasculitis disease?Urticarial vasculitis is uncommon. There are no well researched estimates of how frequent it is.
Who gets Urticarial vasculitis? Both males and females may be affected. Urticarial vasculitis may be associated with a number of diseases. Hypocomplementemic urticarial vasculitis especially is associated with rheumatologic diseases such as systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. Some cancers including leukemias and solid cancers such as colon cancer, pancreas cancer and others also may cause urticarial vasculitis. In addition, certain infections like hepatitis B and C can cause this form of vasculitis, as can some drugs, including antibiotics, ACE inhibitors used for treating high blood pressure, and certain diuretics. Still, the cause of most cases of urticarial vasculitis is unknown.
Symptoms The most common symptoms are hives in the skin, which can cause itching, pain and a feeling of burning of the skin. The skin patches often are red rimed with white centers, and unlike common hives, may have petechia, or bleeding under the skin. The patches can be present for days, and can leave behind skin discoloration as they heal.
Some patients may also have systemic symptoms, meaning symptoms other than in the skin. Urticarial vasculitis may cause fevers, joint and abdominal pain, shortness of breath, swollen lymph glands, and sometimes even causes injury to vital organs including the gut, lungs and kidneys.
DiagnosisUrticarial vasculitis is diagnosed on the basis of characteristic patches in the skin. Biopsy of the skin may be needed to be sure of the diagnosis. The results of the biopsy can show inflammation in the skin and damage of small blood vessels with white blood cells.
Because urticarial vasculitis can be associated with a number of different diseases, it is often necessary to perform examinations to determine if an underlying disease such as lupus erythematosus or cancer is present. Because this form of vasculitis can affect vital organs such as the kidneys, other tests, for example of kidney function and chest radiographs may be needed, especially when the blood levels of complement are low.
Treatmentegardless of the cause, urticarial vasculitis is a treatable condition. The 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 such cases, 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, daps one, and chemotherapies like azathioprine or cyclophosphamide. Treatment may be intermittent, although it is not uncommon that patients need treatment for several years.
What are the potential complications of Urticarial vasculitis?The most common serious complications are skin pigmentation, and occasionally skin ulcers and damage to organs such as the lungs, eyes and kidneys.
What is the prognosisof Urticarial vasculitis?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 the urticarial vasculitis is due to a disease such as lupus or cancer, the prognosis is often governed by the prognosis of the underlying disease.
What is new in Urticarial vasculitis?Progress is being made in understanding how urticarial vasculitis comes about, and how to better treat it.
The Vasculitis Foundation gratefully acknowledges Dr. Eric L. Matteson from the Mayo Clinic, Rochester,MN, for his expertise and contribution in compiling this information. This brochure was made possible by an educational grant from Genentech, Inc., and Biogen Idec.
Urticarial Vasculitis
Urticarial vasculitis is a form of vasculitis that affects the skin, causing wheals or hives and/or red patches. Unlike common hives, these eruptions are actually due to vasculitis of small blood vessels.
There are two types of urticarial vasculitis. One has normal levels of proteins called complements, and the other can have low levels of these complement proteins (so-called hypocomplementemic vasculitis).
