Hunziker T, Nydegger U E, Späth P J, Gerber H A, Hess M, Wiesmann U, Krebs A
Arch Dermatol Res. 1985;277(4):299-303. doi: 10.1007/BF00509084.
A patient suffering from severe pemphigus vulgaris was treated using large-volume plasma exchange in combination with an immunosuppressive regimen. As some recent reports have shown evidence that polyclonal, polyspecific human IgG in high doses through the i.v. route (IGIV) protect target platelets in idiopathic thrombocytopenic purpura from attack by antiplatelet autoantibodies and/or immune complexes, we also administered IGIV to this pemphigus-vulgaris patient. In order to test the hypothesis that IGIV might protect in vitro-cultured human skin from acantholysis induced by pemphigus antibodies, studies with skin organ cultures were carried out using plasma from another pemphigus-vulgaris patient who had undergone plasma exchange. The preincubation of either the skin explants or the pemphigus plasma with various concentrations of IGIV (ranging from 0.15 to 15 mg/ml in the culture medium) did not prevent acantholysis induced by the pemphigus plasma nor did it inhibit the binding of the specific antibodies visualized by direct immunofluorescence. Thus, the assumption that IGIV may coat the pemphigus antigens on epidermal cells making them inaccessible to pathogenic autoantibodies was not substantiated by our tests in vitro; likewise, the hypothesis of functionally blocking autoantibody activity by means of anti-idiotype effects of IGIV cannot be supported.
一名患有严重寻常型天疱疮的患者接受了大容量血浆置换联合免疫抑制方案的治疗。由于最近一些报告显示，高剂量的多克隆、多特异性人IgG通过静脉途径（IGIV）可保护特发性血小板减少性紫癜中的靶血小板免受抗血小板自身抗体和/或免疫复合物的攻击，我们也对这名寻常型天疱疮患者给予了IGIV。为了验证IGIV可能保护体外培养的人皮肤免受天疱疮抗体诱导的棘层松解这一假设，我们使用另一名接受过血浆置换的寻常型天疱疮患者的血浆进行了皮肤器官培养研究。将皮肤外植体或天疱疮血浆与不同浓度的IGIV（培养基中浓度范围为0.15至15mg/ml）预孵育，既不能预防天疱疮血浆诱导的棘层松解，也不能抑制直接免疫荧光显示的特异性抗体的结合。因此，我们的体外试验并未证实IGIV可能覆盖表皮细胞上的天疱疮抗原，使其无法被致病性自身抗体识别这一假设；同样，IGIV通过抗独特型效应功能性阻断自身抗体活性的假设也不能得到支持。
