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Genetic and Rare Diseases Information Center (GARD)

Hypocomplementemic urticarial vasculitis syndrome

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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


  1. Filosto M, Cavallaro T, Pasolini G, Broglio L, Tentorio M, Cotelli M, Ferrari S, Padovani A. J Neurol Sci. 2009; Accessed 7/30/2009.
  2. Habif: Clinical Dermatology, 4th edition. Mosby; 2004;
  3. Mehregan D, Hamzavi I. Urticarial Vasculitis. eMedicine. 2007; Accessed 7/30/2009.
  4. Goldman: Cecil Medicine, 23rd edition. Saunders; 2007;
  5. Firestein: Kelly's Textbook of Rheumatology, 8th edition. Saunders; 2008;
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