McDuffie hypocomplementemic urticarial vasculitis. Two cases and review ofthe literature

Introduction. - Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We rep ort two new cases. Exegesis. - Case 1: a 41-year-old woman presented in 1994 with inflammatory polyarthralgia, diffuse urticaria, fever, and weight loss. Biology showed proteinuria, positive rheumatoid factor with hypocomplementemia and negativ e immunological tests. Skin and renal biopsies showed leukocytoclastic vasc ulitis and extramembranous glomerulopathy, respectively Outcome within ster oid therapy was marked by alternating clinical improvement and relapses. Ca se 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis , diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays an d CT scan showed interstitial fibrosis and echocardiography revealed perica rditis. Biology showed positive rheumatoid factor with hypocomplementemia a nd negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vas culitis. Corticosteroids and cyclophosphamide improved the patient's condit ion. McDuffie HUV is a disease with varied systemic manifestations Its exis tence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids. Conclusion. - McDuffie HUV is a defensible entity among urticarial vasculit is because of ifs particular clinical and biological features. (C) 2001 Edi tions scientifiques et medicales Elsevier SAS.