Zeiss C R, Burch F X, Marder R J, Furey N L, Schmid F R, Gewurz H
Am J Med. 1980 Jun;68(6):867-75. doi: 10.1016/0002-9343(80)90216-8.
We describe four new patients with a unique syndrome of persistent urticaria, with leukoclastic angiitis, severe angioedema, occasional life-threatening laryngeal edema, arthritis, arthralgia, neurologic abnormalities and pronounced persistent hypocomplementemia. The complement abnormalities involved markedly reduced levels of the Clq subunit of the first component of complement (Cl) in the presence of near normal levels of Clr and Cls subunits of Cl; modest to marked depletion of the fourth component of complement (C4), the second component of complement (C2) and the third component of complement (C3); and normal levels of the fifth through ninth components of complement (C5 through C9) and properdin factors B and D. A striking serologic abnormality found in all patients was the presence of low molecular weight (7S) proteins which precipitated with Clq in agarose gels; these previously were shown to be comprised at least in part of immunoglobulin G. The present experience is offered to help to define the clinical, histopathologic and serologic characteristics of this entity, designated hypocomplementemic vasculitic urticarial syndrome, and to emphasize its distinctiveness and prevalence.
我们描述了4例患有独特综合征的新患者，该综合征表现为持续性荨麻疹，伴有白细胞破碎性血管炎、严重血管性水肿、偶发危及生命的喉水肿、关节炎、关节痛、神经异常以及明显的持续性补体低下。补体异常包括：在补体第一成分（C1）的C1q亚单位水平显著降低的同时，C1的C1r和C1s亚单位水平接近正常；补体第四成分（C4）、第二成分（C2）和第三成分（C3）有中度至显著消耗；补体第五至第九成分（C5至C9）以及备解素因子B和D水平正常。在所有患者中发现的一个显著血清学异常是存在低分子量（7S）蛋白，这些蛋白在琼脂糖凝胶中可与C1q沉淀；先前已证明这些蛋白至少部分由免疫球蛋白G组成。本文提供的经验有助于明确这一被称为低补体血症性血管炎性荨麻疹综合征的疾病的临床、组织病理学和血清学特征，并强调其独特性和患病率。
