• Dtsch. Med. Wochenschr. · Aug 2000

    Case Reports Comparative Study

    [Hypocomplementemic urticarial vasculitis syndrome].

    • R Sessler, G Hasche, and C J Olbricht.
    • Zentrum für Innere Medizin, Abteilung für Nieren- und Hochdruckkrankheiten, Katharinenhospital, Stuttgart.
    • Dtsch. Med. Wochenschr. 2000 Aug 25;125(34-35):1003-6.

    History And Admission FindingsA 40-year-old woman was admitted with recurrent membranoproliferative glomerulonephritis. For many years she had been suffering from joint pains and attacks of angio-oedema of the upper and lower lip. Physical examination was unremarkable except for marked oedema and urticaria of the trunk and limbs.InvestigationsSkin biopsy revealed urticaria with vasculitis. Complement fractions C3, C4 and C1q were reduced, and C1q antibodies were demonstrated.Diagnosis, Treatment And CourseThe findings of urticaria with vasculitis and reduced complement levels as cardinal symptoms together with joint pains, glomerulonephritis and C1q antibodies as minor symptoms were diagnostic of hypocomplementaemic vasculitic urticaria syndrome. The urticaria disappeared immediately when immunosuppressive treatment with prednisolone and cyclophosphamide was started, and the nephritic signs regressed.ConclusionDifferentiation of a vasculitic and a non-vasculitic form of chronic urticaria provides an initial step in delineating the underlying disease. Severe systemic disease such as collagen disease or vasculitis is not rare in vasculitic urticaria and requires long-term immunosuppression.

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