The objective of this study was to investigate the frequency and functionality of DCs and its associated stimulatory and inhibitory markers in the pathogenesis of PV Active PV patients (n = 30) having both skin and oral lesions, and 30 healthy controls were recruited in the study. The frequency of DCs was determined by flow cytometry followed by the primary culture by using recombinant IL-4 (250 IU/ml) and GM-CSF (600 IU/ml). The culture supernatant was used for ELISA. RNA was isolated from sorted DCs and used for the mRNA expression of DC-associated stimulatory (CD40 and CD80) and inhibitory (PSGL1 and ILT3) markers. Tissue localization of Langerhans cells was done by immunohistochemistry. In this study, altered frequency of myeloid DC (mDC) and plasmacytoid DC (pDC) was seen in the circulation of PV patients. The primary culture of patient-derived DCs showed anomalous cytokine profiling. In the culture supernatant of DCs, elevated levels of TNF-ɑ and IL-12 were detected in PV patients. Meanwhile, reverse trend was found in the case of IFN-ɑ and IL-10 cytokine levels. Similarly, a discrepancy in the expression of DC-associated stimulatory (CD40 and CD80) and inhibitory (PSGL1 and ILT3) markers suggested their possible involvement in the immunopathogenesis of PV. An elevated number of tissue localizing Langerhans cells was also observed in the perilesional skin. This study indicates the distorted frequency and functionality of DCs in the immunopathogenesis of PV. Targeting these functional markers in the future may generate novel therapeutic options for better management of PV.

本研究的目的是调查 DCs 及其相关刺激和抑制标志物在 PV Active PV 患者发病机制中的频率和功能（n = 30) 有皮肤和口腔病变，并且在研究中招募了 30 名健康对照。DCs 的频率通过流式细胞术测定，然后使用重组 IL-4 (250 IU/ml) 和 GM-CSF (600 IU/ml) 进行原代培养。培养上清液用于ELISA。RNA 从分选的 DC 中分离出来，用于 DC 相关刺激（CD40 和 CD80）和抑制（PSGL1 和 ILT3）标记的 mRNA 表达。朗格汉斯细胞的组织定位通过免疫组织化学完成。在这项研究中，在 PV 患者的循环中观察到髓样 DC (mDC) 和浆细胞样 DC (pDC) 的频率改变。源自患者的 DC 的原代培养显示出异常的细胞因子分析。在 DC 的培养上清液中，PV 患者中检测到 TNF-ɑ 和 IL-12 水平升高。同时，在 IFN-ɑ 和 IL-10 细胞因子水平的情况下发现了相反的趋势。同样，DC 相关刺激（CD40 和 CD80）和抑制（PSGL1 和 ILT3）标记表达的差异表明它们可能参与 PV 的免疫发病机制。在病灶周围皮肤中也观察到定位朗格汉斯细胞的组织数量增加。该研究表明 DCs 在 PV 免疫发病机制中的频率和功能失真。未来针对这些功能性标志物可能会产生新的治疗选择，以更好地管理 PV。在病灶周围皮肤中也观察到定位朗格汉斯细胞的组织数量增加。该研究表明 DCs 在 PV 免疫发病机制中的频率和功能失真。未来针对这些功能性标志物可能会产生新的治疗选择，以更好地管理 PV。在病灶周围皮肤中也观察到定位朗格汉斯细胞的组织数量增加。该研究表明 DCs 在 PV 免疫发病机制中的频率和功能失真。未来针对这些功能性标志物可能会产生新的治疗选择，以更好地管理 PV。