Hypocomplementemic urticarial vasculitis syndrome

Title

Summary Summary

Hypocomplementemic urticarial vasculitis (HUV) is a rare form of cutaneous small-vessel vasculitis characterized by recurrent episodes of urticaria and painful, tender, burning or itchy skin lesions, often associated with extracutaneous involvement but usually with no significant peripheral nerve damage.^[1] Patients with this condition are likely to have systemic involvement, including angioedema, arthralgias, pulmonary disease, abdominal or chest pain, fever, renal disease, and episcleritis.^[2]^[3] Hypocomplementemic urticarial vasculitis is thought be an autoimmune response involving a specific region of complement 1 (C1).^[2] It can present as or precede a syndrome that includes obstructive pulmonary disease , uveitis, systemic lupus erythematous (SLE), Sjögren's syndrome, or cryoglobulinemia (which is closely linked with hepatitis B or hepatitis C virus infection).^[2]^[3]^[4] Some cases of hypocomplementemic urticarial vasculitis respond to therapies commonly used for the treatment of SLE, including low-dose prednisone, hydroxychloroquine, dapsone, or other immunomodulatory agents.^[5]

Last updated: 7/30/2009

Diagnosis Diagnosis

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.^[6] 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:^[6]

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.^[6]

Differential diagnoses for HUV syndrome include, Schnitzler's syndrome, Cogan's syndrome, and Muckle-Wells syndrome.^[6]

Last updated: 3/6/2013

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are studying or have studied Hypocomplementemic urticarial vasculitis syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Healthcare Resources

If your physician would like to review your case with a vasculitis specialist, the Vasculitis Foundation lists vasculitis medical consultants that may be able to speak with your healthcare provider. Click on the link above to learn more.

Financial Resources

The Social Security Administration has included this condition in their Compassionate Allowances Initiative. This initiative speeds up the processing of disability claims for applicants with certain medical conditions that cause severe disability. More information about Compassionate Allowances and applying for Social Security disability is available online.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
PubMed is a searchable database of medical literature and lists journal articles that discuss Hypocomplementemic urticarial vasculitis syndrome. Click on the link to view a sample search on this topic.

News & Events News & Events

NCATS Co-Sponsored Conferences

Workshop on Systemic Vasculitis: Future Research Directions Monday, September 15, 1997
Location: NIH Campus, Bethesda, MD
Contact: Dr. Howard Dickler(301) 496-7104

Co-funding Institute(s): National Institute of Allergy and Infectious Diseases

Other Conferences

All Star Vasculitis Patient Symposium
July 30, 2010 - August 1, 2010
Location: Westin Long Beach Hotel in Long Beach, California
Description: Topics will cover all the types of vasculitis and will concentrate on the advances in medical treatments, research, and quality of life of patients. The meeting attracts vasculitis patients, family members, and medical vasculitis experts in the world.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new 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? See answer

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References References

Filosto M, Cavallaro T, Pasolini G, Broglio L, Tentorio M, Cotelli M, Ferrari S, Padovani A. J Neurol Sci. 2009; http://www.ncbi.nlm.nih.gov/pubmed/19375087. Accessed 7/30/2009.
Habif: Clinical Dermatology, 4th edition. Mosby; 2004;
Mehregan D, Hamzavi I. Urticarial Vasculitis. eMedicine. 2007; http://emedicine.medscape.com/article/1085087-overview. Accessed 7/30/2009.
Goldman: Cecil Medicine, 23rd edition. Saunders; 2007;
Firestein: Kelly's Textbook of Rheumatology, 8th edition. Saunders; 2008;
Brewer JD, Davis MDP. Urticarial vasculitis. In: Basow, DS. UpToDate. Waltham, MA: UpToDate; 2012;