Pan Dan, Wang Jiongke, Cai Luyao, Huang Mei, Han Qi, Zheng Zhijian, Zeng Xin, Chen Qianming, Ai Ruixue, Zhou Yu
State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, Chinese Academy of Medical Sciences Research Unit of Oral Carcinogenesis and Management, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.
State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, Department of Oral Pathology, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.
J Clin Med. 2025 Jun 12;14(12):4198. doi: 10.3390/jcm14124198.
Pyodermatitis-pyostomatitis vegetans (PPV) is a rare, chronic inflammatory mucocutaneous disorder. However, the etiology of PPV remains controversial. : A review of online PPV case studies from PubMed, Wanfang database, Web of Science, and books has been performed. Comparative analysis of langerin expression has been conducted to verify the hypothesis summarized from the literature review by Immunohistochemistry (IHC). : A total of 63 patients were analyzed across 5 reviews, 44 case reports, and 1 book chapter. Our findings revealed distinct immunological alterations in PPV patients. Innate immunity was upregulated, marked by increased neutrophil and eosinophil counts and enhanced macrophage activity. Adaptive immunity was suppressed, with reduced dendritic cell (DC) numbers and activity and diminished adaptive immune responses. We hypothesize that langerin was a critical factor, contributing to adaptive immune suppression and a compensatory innate immune hyperactivation. : We propose the hypothesis that langerin expression on Langerhans cells (LCs) plays a pivotal role in PPV pathogenesis by shifting the immune balance toward innate hyperactivation at the expense of adaptive immunity.
增殖性皮炎-增殖性口炎（PPV）是一种罕见的慢性炎症性黏膜皮肤疾病。然而，PPV的病因仍存在争议。：我们对来自PubMed、万方数据库、科学网的在线PPV病例研究以及书籍进行了综述。通过免疫组织化学（IHC）对朗格汉斯蛋白表达进行了比较分析，以验证从文献综述中总结出的假设。：共分析了5篇综述、44篇病例报告和1章书籍中的63例患者。我们的研究结果揭示了PPV患者存在明显的免疫改变。固有免疫上调，表现为中性粒细胞和嗜酸性粒细胞计数增加以及巨噬细胞活性增强。适应性免疫受到抑制，树突状细胞（DC）数量和活性减少，适应性免疫反应减弱。我们假设朗格汉斯蛋白是一个关键因素，导致适应性免疫抑制和代偿性固有免疫过度激活。：我们提出假设，即朗格汉斯细胞（LC）上的朗格汉斯蛋白表达通过将免疫平衡转向以适应性免疫为代价的固有免疫过度激活，在PPV发病机制中起关键作用。
