Urticarial Vasculitis

Elena Borzova, Clive E. H. Grattan


Urticarial vasculitis is a rare disease that needs to be differentiated from chronic spontaneous urticaria. The illness is characterized clinically by urticarial lesions with histological evidence of leucocytoclastic vasculitis. It can lead to substantial morbidity if associated with hypocomplementaemia and systemic involvement. The diagnosis relies on lesional skin biopsy and may present challenges for both clinicians and histopathologists. Investigation for an underlying infection or an autoimmune‐, neoplastic‐ or drug‐related aetiology may reveal a cause but many cases remain unexplained after investigation. Management of urticarial vasculitis can be difficult and includes H1 antihistamines, non‐steriodal anti‐inflammatory drugs, oral corticosteroids, antimalarials and immunosuppressive agents, but evidence of efficacy from controlled studies is needed.
Keywords urticarial vasculitis, urticarial venulitis, McDuffie syndrome, leukocytoclasis, hypocomplementaemic urticarial vasculitis syndrome, Schnitzler syndrome


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