Incessant relentless ITCHING
UV that causes HIVES & wheals
& Skin that burns and bleeds
Add to our Lupus & Chronic Needs-
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.
What causes Urticarial 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.
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 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 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.
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.
It Happens Like THIS:
What is a Hypersensitivity Reaction?
In our case, when we are exposed to Ultraviolet – Antibodies & Immune Complexes Are Triggered & Cause a Hypersensitivity Type 3 Reaction:
My Personal Experience With Urticarial Vasculitis
Those rashes up there are all mine. They all ITCHED horribly, a deep DEEP intractable painful burning type itch that could not be relieved. Like a friend said, like poison ivy times 100. On several occasions my arm tingled and I had some muscle weakness. I get mine with hard nodules under my skin- you can feel them, hard lumps right on the itch that hurt when you touch or press them.
Mine are always activated by UV exposure. I live in the desert where the summer months are killer-and I spend most of my time indoors to avoid lupus flare ups. But it always gets me! I have double blackout drapes on my windows and that makes me pretty safe indoors-but noone can stay indoors ALL the time!
I almost always flare with the urticaria combined with uveitis (inflammation in the iris of eye), nose sores & mouth sores and my malar rash becomes prominent. Sometimes I get low grade fevers and fatigue with the reaction.
My rashes respond pretty well to betamethazone steroid cream and usually 40mg of prednisone for a few days with an immediate taper down to 0. I take plaquenil and cellcept to control my lupus on a daily basis and it works well for me. I’m extremely lucky that my rashes usually start healing after 24 to 48 hours. Sometimes I end up with discolored skin (see above photo on left). Sometimes I end up with skin so hard on my face-it looks like I’m getting a chemical peel! The skin underneath is usually fine.
I’ve had rash reactions that don’t stop at lupus or urticaria- I’ve had autoimmune pemphigus rashes like this one on the back of my neck- and I get malar rashes like most lupus patients do! But I always recover! 😀
If you have any questions- feel free to ask! I’m happy to help!
Best to You! JJ
More Urticaria Vasculitis Info-explained by Dr Donald Thomas, author of “The Lupus Encyclopedia “. You can follow him on twitter at @lupuscyclopedia
Urticarial vasculitis is a less common form of vasculitis seen in people who have lupus. The areas of vasculitis look similar to hives (the medical term for hives is “urticaria”).
The skin lesions of urticarial vasculitis are typically slightly raised areas of skin that are tender to the touch. When they heal, they often leave pigment changes on the skin. Usually a skin biopsy is required to diagnose urticarial vasculitis accurately.
Doctors usually check SLE patients who develop vasculitis for substances in the blood called cryoglobulins. “Cryo-”comes from the Greek word for “cold,”while “globulins”refer to antibodies. These particular antibodies precipitate and clump together with cooler temperatures.
“Precipitation”is a scientific term meaning that a substance in a liquid solidifies; for example, if you warm up a pan of water and pour in a bunch of salt, the salt dissolves in the water. When the water then cools down in the refrigerator, salt begins precipitating out of the water as salt crystals on the edges of the pan. What happens in people who have cryoglobulinemia (cryoglobulins in their blood) is that the cryoglobulins can clump together and precipitate in parts of the body that are cooler than other parts.
This may occur in the cooler skin of the legs, causing bruised appearing areas and inflammation of the blood vessels (vasculitis). Doctors treat this condition with high doses of steroids along with immunosuppressants. Sometimes doctors have to use a special type of treatment called plasmapheresis, which removes the cryoglobulins from the blood.
Sometimes vasculitis of the skin can occur along with vasculitis in other organs of the body such as the kidneys.
Therefore, if you develop the symptoms of vasculitis, it is important to see your doctor immediately to make sure this is not the case.
Most of the time, cutaneous vasculitis occurs by itself, without involvement of internal organs, but it is important that your doctor checks you thoroughly, especially the first time it happens.