Pemphigus encompasses a group of life-threatening blistering diseases of the skin in which loss of adhesion between keratinocytes is caused by autoantibodies (Ab) against desmogleins (Dsg) 1 and 3. There is major interest in characterizing autoreactive T cells that are presumably critical for the induction and regulation of Ab production. In a recent study, peripheral Dsg3-reactive T helper (Th) cells from patients with acute onset, chronic active and remittent pemphigus vulgaris (PV) were quantitated by MACS secretion assay. Dsg3-reactive Th2 cells were detected at similar frequencies in all the studied PV patients while the number of autoreactive Th1 cells exceeded those of the Th2 cells in chronic active PV. Noteworthy, healthy carriers of the PV-associated HLA class II alleles, DRbeta1*0402 and DQbeta1*0503, exhibited exclusively Th1 reactivity against Dsg3. The titers of Dsg3-reactive IgG were directly related to the ratio of autoreactive Th1/Th2 cells. Moreover, T cell recognition of Dsg3 was restricted by these HLA class II alleles. These findings strongly suggest that (1) Dsg3-reactive Th2 cells are restricted to PV, (2) distinct HLA class II alleles are critical for T cell recognition of Dsg3, and (3) Ab production is associated with both, Th1 and Th2 cells.

中文翻译：

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针对天疱疮中的桥粒糖蛋白的T细胞自身免疫，天疱疮是一种自身抗体介导的皮肤大疱性疾病。

天疱疮包括一组威胁生命的皮肤水疱性疾病，其中角质形成细胞之间的粘附力丧失是由针对桥粒糖蛋白（Dsg）1和3的自身抗体（Ab）引起的。抗体生产的诱导和调控。在最近的一项研究中，通过MACS分泌测定对来自急性发作，慢性活动和易感寻常性天疱疮（PV）患者的外周Dsg3反应性T辅助（Th）细胞进行了定量。在所有研究的PV患者中，以相似的频率检测到Dsg3反应性Th2细胞，而在慢性活动性PV中，自反应性Th1细胞的数量超过了Th2细胞的数量。与PV相关的HLA II类等位基因DRbeta1 * 0402和DQbeta1 * 0503的值得注意的健康载体，仅表现出对Dsg3的Th1反应性。Dsg3反应性IgG的滴度与自身反应性Th1 / Th2细胞的比例直接相关。此外，Dsg3的T细胞识别受到这些HLA II类等位基因的限制。这些发现强烈表明（1）Dsg3反应性Th2细胞仅限于PV，（2）不同的HLA II类等位基因对Tsg3对Tsg3的识别至关重要，（3）Ab的产生与Th1和Th2细胞都相关。