McDuffie hypocomplementemic urticarial vasculitis. Two cases and review of the literature

• Published in: La revue de medecine interne 2001-01, Vol.22 (1), p.70
• Main Authors: el Maghraoui, A, Abouzahir, A, Mahassine, F, Tabache, F, Bezza, A, Ghafir, D, Ohayon, V, Archane, M I
• Format: Article
• Language: fre
• Subjects: Adult; Cyclophosphamide - therapeutic use; Diagnosis, Differential; Female; Glomerulonephritis, Membranoproliferative - diagnosis; Glomerulonephritis, Membranoproliferative - drug therapy; Glomerulonephritis, Membranoproliferative - pathology; Humans; Immunosuppressive Agents - therapeutic use; Steroids - therapeutic use; Treatment Outcome; Urticaria - diagnosis; Urticaria - drug therapy; Urticaria - pathology; Vasculitis, Leukocytoclastic, Cutaneous - diagnosis; Vasculitis, Leukocytoclastic, Cutaneous - drug therapy; Vasculitis, Leukocytoclastic, Cutaneous - pathology
• ISSN: 0248-8663
• DOI: 10.1016/S0248-8663(00)00288-5

Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We report two new cases. 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 negative immunological tests. Skin and renal biopsies showed leukocytoclastic vasculitis and extramembranous glomerulopathy, respectively. Outcome within steroid therapy was marked by alternating clinical improvement and relapses. Case 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis, diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays and CT scan showed interstitial fibrosis and echocardiography revealed pericarditis. Biology showed positive rheumatoid factor with hypocomplementemia and negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vasculitis. Corticosteroids and cyclophosphamide improved the patient's condition. McDuffie HUV is a disease with varied systemic manifestations. Its existence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids. McDuffie HUV is a defensible entity among urticarial vasculitis because of its particular clinical and biological features.