Urticarial Vasculitis

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Urticarial Vasculitis is a variant of cutaneous vasculitis. It is characterised by inflamed and reddened patches or weals on the skin that appear to resemble urticaria, but when the skin is examined closely under a microscope, vasculitis is found (inflamed blood vessels).

This disease is generally classified as two types, normocomplementaemic and hypocomplementaemic (normal or lowered levels of complement proteins found on blood testing). Although both types may be associated with systemic symptoms such as angioedema, abdominal or chest pain, fever, and joint pain, this is more apparent in the hypocomplementaemic form. This form has also been linked to the connective tissue disease systemic lupus erythematosus.

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  • Hives
  • Breathing difficulty
  • Chronic hypocomplementemia
  • Joint pain
  • Arthritis
  • Inflammation of uvea of the eye
  • Episcleritis
  • Recurrent abdominal pain
  • Venulitis of dermid
  • Glomerulonephritis
  • Blood in urine
  • Excess protein in urine
The cause is unknown but it has been associated with the following conditions:
  • Inflammatory connective disorders such as SLE and Sjögren syndrome.
  • Immunoglobulin disorders such as immunoglobulin A and immunoglobulin M monoclonal gammopathies
  • Leukaemia and internal cancers
  • Viral diseases such as hepatitis B, hepatitis C and glandular fever
  • Drug-induced conditions from ACE inhibitors, penicillin, sulfonamides, fluoxetine and thiazides.
  • However, the majority of cases arise from no known cause.

    Patients with normocomplementaemic urticarial vasculitis usually have minimal or no systemic involvement and lesions often resolve on their own over time.

    Treatment is based on systemic effects of the disease and extent of cutaneous involvement. To relieve cutaneous symptoms, antihistamines or non-steroidal anti-inflammatory drugs (NSAIDs) may be used.

    Treatments that may be used in the long-term control of severe cases that may be associated with systemic symptoms include:

  • Dapsone
  • Colchicine
  • Hydroxychloroquine
  • Indometacin/indomethacin (non-steroidal anti-inflammatory)
  • Corticosteroids, e.g. oral prednisone

  • For cases that are resistant to treatment with corticosteroids other immunosuppressive agents such as azathioprine, cyclophosphamide or ciclosporin may be considered.


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