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Hypocomplementemic urticarial vasculitis syndrome
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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]
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 July 30, 2009.
- Habif: Clinical Dermatology, 4th edition. Mosby; 2004.
- Mehregan D, Hamzavi I. Urticarial Vasculitis. eMedicine. http://emedicine.medscape.com/article/1085087-overview. Accessed July 30, 2009.
- Goldman: Cecil Medicine, 23rd edition. Saunders; 2007.
- Firestein: Kelly's Textbook of Rheumatology, 8th edition. Saunders; 2008.
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General Information
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Insurance Issues
- 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.