Katz S I, Hertz K C, Yaoita H
J Clin Invest. 1976 Jun;57(6):1434-41. doi: 10.1172/JCI108413.
Five patients with herpes gestationis, a blistering disease of pregnancy, were studied immunologically. All had in vivo deposition of C3 in a linear band along the basement membrane zone of lesional and normal-appearing skin, the location of early blister formation. Immunoglobulin deposition was more variable, though four patients had evidence of in vivo bound IgG at the same site. A circulating, complement binding herpes gestationis factor was demonstrated in the sera of four of the patients, its concentration unrelated to the activity of clinical disease. Characterization of this factor by sucrose gradient ultracentrifugation, specific absorption studies, and papain digestion indicates that it is an IgG. Evidence exists for involvement of both the classical and alternate complement pathways in vivo, though in vitro studies implicate the classical pathway as the primary route of complement activation. Three offspring were studied, none with clinical involvement; one showed in vivo deposition of C3 at the basement membrane zone of normal skin and a second showed the herpes gestationis factor in cord blood.
对五名妊娠疱疹患者(一种妊娠期水疱性疾病)进行了免疫学研究。所有患者病变皮肤和外观正常皮肤的基底膜带均有C3呈线性条带的体内沉积,这是早期水疱形成的部位。免疫球蛋白沉积情况更具变异性,不过有四名患者在同一部位有体内结合IgG的证据。在四名患者的血清中检测到一种循环的、结合补体的妊娠疱疹因子,其浓度与临床疾病活动无关。通过蔗糖梯度超速离心、特异性吸收研究和木瓜蛋白酶消化对该因子进行表征,表明它是一种IgG。有证据表明体内经典补体途径和替代补体途径均参与其中,不过体外研究表明经典途径是补体激活的主要途径。对三名后代进行了研究,均无临床受累情况;一名在正常皮肤的基底膜带显示有C3的体内沉积,另一名在脐带血中检测到妊娠疱疹因子。