中文摘要：

背景对多重表皮的蛋白质的自身抗体的存在是在 paraneoplastic 天疱疮(PNP ) 的一个重要特征。传播 anti-desmoglein 3 自身抗体，在天疱疮 vulgaris (PV ) 的主要病原的自身抗体，在 PNP 被证明病原。因为 PNP 和 PV 之间的许多临床的差别，我们在 PNP 的致病关于另外的自身抗体的参与推测。Envoplakin (EPL ) 和 periplakin (PPL ) 被大多数 PNP 认出干枯。他们的连接器子域为我们描绘了的中间的 filaments.Methods 的协会高度相应、必要对在 PNP 病人由连接酶的 immunosorbent 试金(ELISA ) 和 immunofluorence 的 sera 和他们的联系肿瘤的 EPL 和 PPL 的连接器子域的自身抗体。我们也从 PNP sera 对 EPL 和 PPL 把净化的自身抗体用于有教养的人的表皮的 keratinocytes ( HEK )，到把房间房间 adhesion.Results 自身抗体的变化与 EPL 和 PPL 作比较被 ELISA 在大多数 PNP 病人检测，并且在肿瘤的移动以后的这些自身抗体的减少粗略地比得上临床的症状的改进。从 PNP 病人的有教养的肿瘤房间分泌了这些自身抗体。为 EPL 和 PPL 的特定的免疫球蛋白受体从 PNP 在肿瘤在 B 淋巴细胞上被发现。而且，从 PNP 净化了 anti-EPL 和 anti-PPL 自身抗体干枯能够分裂对 EPL 和 PPL 的有教养的人的表皮的 keratinocytes.Conclusion 自身抗体可能也在 PNP 是病原的。

英文摘要：

Background The presence of autoantibodies against multiple epidermal proteins is an important feature in paraneoplastic pemphigus （PNP）. Circulating anti-desmoglein 3 autoantibody, the major pathogenic autoantibody in pemphigus vulgaris （PV）, has been proved pathogenic in PNP. Because of many clinical differences between PNP and PV, we speculate about the involvement of other autoantibodies in the pathogenesis of PNP. Envoplakin （EPL） and periplakin （PPL） are recognized by most PNP sera. Their linker subdomains are highly homologous and necessary for the association of intermediate filaments. Methods We characterized the autoantibodies against the linker subdomains of EPL and PPL in PNP patients＇ sera and their associated tumors by enzyme-linked immunosorbent assay （ELISA） and immunofluorence. We also applied the purified autoantibodies against EPL and PPL from PNP sera to cultured human epidermal keratinocytes （HEK）, to evaluate the changes of cell-cell adhesion. Results Autoantibodies against EPL and PPL were detected in most PNP patients by ELISA, and the decrease of these autoantibodies after removal of the tumors was roughly comparable to the improvement of clinical symptoms. Cultured tumor cells from PNP patients secreted these autoantibodies. Specific immunoglobulin receptors for EPL and PPL were found on B lymphocytes in tumors from PNP. Furthermore, purified anti-EPL and anti-PPL autoantibodies from PNP sera were capable of dissociating cultured human epidermal keratinocytes. Conclusion Autoantibodies against EPL and PPL may also be pathogenic in PNP.