Urticarial Vasculitis

Urticarial vasculitis causes itchy, painful hives to form on your skin. It causes inflammation in blood vessels that can affect your skin and other organs throughout your body. A healthcare provider will find treatments to help you manage your symptoms during episodes.

Overview

What is urticarial vasculitis?

Urticarial vasculitis is a rare form of vasculitis that causes itchy, painful hives on your skin.

Vasculitis is any inflammation (swelling) that affects your blood vessels. Blood vessels are channels that carry blood throughout your body. They form a circuit that begins and ends at your heart. If you have vasculitis, your blood vessels swell and stretch. This makes it harder for blood to flow through them.

Urticaria is the medical term for hives. Hives are raised red or discolored bumps (welts) on your skin. A healthcare provider might refer to hives as skin lesions.

Urticarial vasculitis usually affects your skin, but it can also affect blood vessels connected to other areas of your body, including your:

Depending on which symptoms you’re experiencing — and how severe they are — most people with urticarial vasculitis can manage their symptoms with medication.

Visit a healthcare provider if you have hives on your skin that don’t go away or get better in a few days.

Urticarial vasculitis that affects your internal organs can cause life-threatening symptoms. Go to the emergency room or call 911 (or your local emergency number) if you have any of the following symptoms:

How common is urticarial vasculitis?

Urticarial vasculitis is rare. Experts aren’t sure exactly how many people experience it each year. Many people with mild symptoms don’t get it diagnosed. Severe cases are rare enough that it’s hard to estimate how common they are.

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Symptoms and Causes

What are the symptoms of urticarial vasculitis?

Most people with urticarial vasculitis experience periods of symptoms that come and go, called episodes.

Hives on your skin is the most common symptom of urticarial vasculitis. You can develop hives anywhere on your skin during an urticarial vasculitis episode. The hives are usually:

  • Swollen, raised bumps you can see and feel.
  • Itchy.
  • Painful.
  • Red or discolored.

Severe symptoms of urticarial vasculitis are rare, but include:

During an episode of symptoms, you might experience angioedema (swelling in tissue under your skin). After an episode goes away (subsides), the skin where the hives were might look bruised or discolored.

What causes urticarial vasculitis?

Experts aren’t sure what causes urticarial vasculitis.

Vasculitis is an autoimmune disease. Autoimmune diseases are the result of your immune system accidentally attacking your body instead of protecting it.

Urticarial vasculitis can sometimes develop after a heath condition causes inflammation in your body. This is called a trigger. Some people develop urticarial vasculitis with no cause or trigger (developing it idiopathically).

Some triggers of urticarial vasculitis include:

  • Infections.
  • Other autoimmune diseases.
  • As a side effect of some drugs and medications.
  • Some types of cancer.

Autoimmune diseases that trigger urticarial vasculitis

Any autoimmune disease can trigger urticarial vasculitis, especially:

Infections that trigger urticarial vasculitis

Some infections that trigger urticarial vasculitis include:

Urticarial vasculitis risk factors

Anyone can develop urticarial vasculitis. It’s more common in adults older than 30.

Women and people assigned female at birth (AFAB) are more than twice as likely to develop urticarial vasculitis as men and people assigned male at birth (AMAB).

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Diagnosis and Tests

How is urticarial vasculitis diagnosed?

A healthcare provider will diagnose urticarial vasculitis. They’ll look at your skin and ask you about any symptoms you’re experiencing.

Diagnosing urticarial vasculitis is usually part of a differential diagnosis for other conditions. This means your provider will probably use a few tests to determine what’s causing your symptoms before diagnosing you with urticarial vasculitis. Some tests you might need include:

  • Skin biopsy of the hives.
  • Blood tests.
  • Tests that check the function of your affected organs.

You’ll probably need to see a rheumatologist — a healthcare provider who specializes in treating inflammatory diseases.

Your provider might suggest you visit a pulmonologist (a specialist who treats your respiratory system) if you’re having symptoms that affect your breathing.

Management and Treatment

How is urticarial vasculitis treated?

Your provider will suggest treatments that manage the urticarial vasculitis symptoms you’re experiencing. Medications that reduce swelling can help relieve hives and inflammation. You might need:

Your provider will work with you to find a treatment that manages your symptoms. Many people with urticarial vasculitis have other health conditions at the same time. Which other conditions you have can affect which treatments will work for you. Tell your provider about any other health conditions you have and which other medications or treatments you’re currently using.

Ask your provider about any side effects of the medications they recommend to treat urticarial vasculitis. They’ll tell you what to expect and which signs of complications to watch for.

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Is there a cure for urticarial vasculitis?

There’s no cure for urticarial vasculitis, but some people eventually go into remission — long periods of time between episodes of symptoms, usually months or years.

Prevention

How can I prevent urticarial vasculitis?

You can’t prevent urticarial vasculitis. Because experts don’t know what causes it, there’s no way to know if or when you’ll develop it.

Outlook / Prognosis

What can I expect if I have urticarial vasculitis?

You should expect to have urticarial vasculitis for a long time — maybe for the rest of your life. Most people with urticarial vasculitis only ever experience symptoms that affect their skin. But if you have urticarial vasculitis that affects other organs, the damage can be life-threatening.

Even if you’re in remission, there’s always a chance you can have a new urticarial vasculitis episode.

Depending on which other health conditions you have, your healthcare provider will tell you what to expect. Visit a provider regularly so they can monitor any changes in your symptoms. You might need regular screenings and tests to make sure the urticarial vasculitis hasn’t spread or changed.

Living With

How do I take care of myself?

Monitor your symptoms, and keep track of any changes you notice. The longer you live with urticarial vasculitis, the better you’ll become at recognizing the early signs of an episode. Follow the treatment plan you worked on with your healthcare provider. Don’t hesitate to visit them if you think something isn’t working (or isn’t as effective as it used to be).

When should I see my healthcare provider?

See your healthcare provider if you notice new or changing symptoms of urticarial vasculitis. Talk to your provider if your episodes are more severe or happening more frequently.

Your provider will tell you how often you’ll need regular follow-up appointments or additional screenings.

Go to the emergency room or call 911 (or your local emergency number) if you’re experiencing any of the following symptoms:

  • You can’t breathe.
  • You feel like you’re having a heart attack.
  • Your vision suddenly gets worse or you have vision loss.

What questions should I ask my doctor?

  • What triggered the urticarial vasculitis?
  • Will it affect organs other than my skin?
  • Which tests will I need?
  • Which treatments will help manage my symptoms?
  • What are the side effects of these treatments?
  • How often will I need follow-up appointments or screenings?

Additional Common Questions

What is the difference between urticaria and urticarial vasculitis?

Urticaria is the medical term for hives. They look like red or discolored bumps on your skin. Healthcare providers describe other health conditions as urticarial if that condition causes hives as a symptom. That’s where urticarial vasculitis gets its name — it’s a form of vasculitis that causes hives to appear on your skin.

Is there a link between urticarial vasculitis and pregnancy?

Experts don’t know for certain what causes urticarial vasculitis. That’s why it’s hard to know which conditions or changes in your body can or can’t trigger it.

Some autoimmune diseases can be triggered in a person’s body when they’re pregnant. Talk to your provider if you notice any changes on your skin while you’re pregnant — especially if you notice hives or a rash you’ve never had before.

A note from Cleveland Clinic

Urticarial vasculitis is a rare form of vasculitis. It usually only affects your skin and makes you have episodes of itchy, painful hives. But it can also affect other organs and systems in your body. Talk to your provider about the best ways to manage your symptoms during an episode of urticarial vasculitis.

Living with a chronic condition can be hard. You might feel tired, exhausted and frustrated. These are valid, real feelings. Talk to your provider or a mental health professional about support groups and other resources that can help you if you’re feeling overwhelmed.

Medically Reviewed

Last reviewed by a Cleveland Clinic medical professional on 08/17/2023.

Learn more about our editorial process.

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