[Hypocomplementemic urticarial vasculitis syndrome]

Abstract

History and admission findings: A 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.

Investigations: Skin biopsy revealed urticaria with vasculitis. Complement fractions C3, C4 and C1q were reduced, and C1q antibodies were demonstrated.

Diagnosis, treatment and course: The 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.

Conclusion: Differentiation 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.