España A, Diaz L A, Mascaró J M, Giudice G J, Fairley J A, Till G O, Liu Z
Department of Dermatology, Medical College of Wisconsin, Milwaukee 53226, USA.
Clin Immunol Immunopathol. 1997 Oct;85(1):83-9. doi: 10.1006/clin.1997.4407.
Pemphigus foliaceus (PF) is a dermatosis characterized by subcorneal vesicles and pathogenic IgG autoantibodies against desmoglein 1. PF IgG passively transferred into neonatal mice induces a blistering disease that duplicates the key findings of PF. In this study we have used this animal model to investigate the role of complement and IgG valence in triggering blister formation. In the passive transfer experiments, we found that PF IgG, as well as the F(ab')2 and Fab fragments, was capable of inducing the typical subcorneal blistering disease in both complement-deficient and complement-sufficient mice. Moreover, the disease activity in these mice correlated well with the dose of IgG or its proteolytic fragments injected in the animals. We conclude that neither complement activation nor IgG-mediated cell surface antigen crosslinking is required for the induction of acantholysis in the experimental PF model.
落叶型天疱疮（PF）是一种以角层下水疱和针对桥粒芯糖蛋白1的致病性IgG自身抗体为特征的皮肤病。将PF IgG被动转移到新生小鼠体内可诱发一种水疱性疾病，该疾病重现了PF的关键特征。在本研究中，我们使用这种动物模型来研究补体和IgG价态在引发水疱形成中的作用。在被动转移实验中，我们发现PF IgG以及F(ab')2和Fab片段能够在补体缺陷和补体充足的小鼠中诱发典型的角层下水疱性疾病。此外，这些小鼠的疾病活动与注射到动物体内的IgG或其蛋白水解片段的剂量密切相关。我们得出结论，在实验性PF模型中，棘层松解的诱导既不需要补体激活，也不需要IgG介导的细胞表面抗原交联。
