Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Hypocomplementemic urticarial vasculitis syndrome


See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview


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

References

  1. 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.
  2. Habif: Clinical Dermatology, 4th edition. Mosby; 2004;
  3. Mehregan D, Hamzavi I. Urticarial Vasculitis. eMedicine. 2007; http://emedicine.medscape.com/article/1085087-overview. Accessed 7/30/2009.
  4. Goldman: Cecil Medicine, 23rd edition. Saunders; 2007;
  5. Firestein: Kelly's Textbook of Rheumatology, 8th edition. Saunders; 2008;
Your Questions Answered
by the Genetic and Rare Diseases Information Center

1 question(s) from the public on Hypocomplementemic urticarial vasculitis syndrome have been answered. See questions and answers. You can also submit a new question.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • 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.

Insurance Issues