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Genetic and Rare Diseases Information Center (GARD)

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Hypocomplementemic urticarial vasculitis syndrome

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Your Question

What tests are used for hypocomplementemic urticarial vasculitis? I noticed while reading your site that hypocomplementemic urticarial vasculitis seemed to fit my symptoms.  Are there tests specifically for this condition as opposed to another type of urticaria angioedema?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How is hypocomplementemic urticarial vasculitis (HUV) diagnosed? What kind of tests are required?

A diagnosis of hypocomplementemic urticarial vasculitis (HUV) syndrome is supported by findings from varied tests, such as skin biopsy, blood tests, physical and eye examinations, and urinalysis and kidney imaging studies (when glomerulonephritis is suspected). 

People with HUV syndrome have hives (urticaria) for at least six months and low levels of proteins (complement) in the blood.[1] Complement levels can be determined through a blood test. Click here to visit the American Association for Clinical Chemistry's Web site Lab Tests Online to learn more about this test. 

In addition to these major criteria, people with HUV syndrome must also have at least two of the following minor criteria:[1]

Inflammation in the small veins of the dermis (diagnosed by biopsy)
Joint pain or arthritis
Mild glomerulonephritis
Inflammation in the eye (uvea or episclera)
Recurrent abdominal pain
The presence of anti-C1q antibodies (this test is not widely available)

Some people have urticarial vasculitis and low complement levels (hypocomplementemia), but do not meet diagnostic criteria for HUV syndrome. These individuals may be diagnosed as having HUV (where symptoms are limited to the skin), versus HUV syndrome.[1]

Differential diagnoses for HUV syndrome include, Schnitzler's syndrome, Cogan's syndrome, and Muckle-Wells syndrome.[1]

Last updated: 3/6/2013

  • Brewer JD, Davis MDP. Urticarial vasculitis. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2012;