el Maghraoui A, Abouzahir A, Mahassine F, Tabache F, Bezza A, Ghafir D, Ohayon V, Archane M I
Service de médecine B, hôpital militaire d'instruction Mohamed V, Rabat, Maroc.
Rev Med Interne. 2001 Jan;22(1):70-4. 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.
麦克达菲所描述的低补体血症性荨麻疹性血管炎（HUV）是一种近来在血管炎中被独立出来的罕见病症。我们报告两例新病例。
病例1：一名41岁女性于1994年出现炎症性多关节痛、弥漫性荨麻疹、发热及体重减轻。实验室检查显示蛋白尿、类风湿因子阳性伴低补体血症，免疫学检查阴性。皮肤和肾脏活检分别显示白细胞破碎性血管炎和膜性外肾小球病变。类固醇治疗期间病情表现为临床症状交替改善与复发。病例2：一名39岁女性于1994年出现炎症性多关节炎、弥漫性荨麻疹、雷诺现象、咳嗽及呼吸困难。胸部X光和CT扫描显示间质性纤维化，超声心动图显示心包炎。实验室检查显示类风湿因子阳性伴低补体血症，抗核抗体阴性。皮肤活检显示白细胞破碎性血管炎。皮质类固醇和环磷酰胺改善了患者病情。麦克达菲HUV是一种具有多种全身表现的疾病。其存在仍受到一些作者的质疑。治疗仍为经验性的，取决于临床特征。主要基于皮质类固醇。
由于其特殊的临床和实验室特征，麦克达菲HUV在荨麻疹性血管炎中是一种可明确界定的病症。
