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C4d表达在大疱性类天疱疮诊断中的作用:一项临床与组织病理学研究

The role of C4d expression in the diagnosis of bullous pemphigoid: a clinical and histopathological study.

作者信息

Kaya Şeyhmus, Yüceer Ramazan Oğuz, Tosun Mustafa, Mustafaeva Reikhan

机构信息

Faculty of Medicine, Department of Pathology, Sivas Cumhuriyet University, Sivas, Turkey.

Faculty of Medicine, Department of Dermatology, Sivas Cumhuriyet University, Sivas, Turkey.

出版信息

Arch Dermatol Res. 2025 Mar 17;317(1):569. doi: 10.1007/s00403-025-04031-2.

Abstract

Inflammatory processes play a crucial role in both autoimmune and non-autoimmune skin diseases. The complement system contributes to these processes, with both well-established and yet-to-be-confirmed effects. The relationship between C4d, an intermediate product of the complement system, and skin diseases remains an area of ongoing investigation. While the presence and role of C4d in autoimmune and non-autoimmune skin diseases have been explored using immunohistochemical methods, the topic remains inconclusive. Bullous pemphigoid is an autoimmune skin disorder characterized by subepidermal blistering, predominantly affecting the elderly population. This study aims to evaluate the potential of C4d as an alternative immunohistochemical marker to the gold-standard diagnostic method, direct immunofluorescence, in diagnosing Bullous pemphigoid. Additionally, C4d staining patterns were analyzed in correlation with clinical data to assess its prognostic significance. In this retrospective study, the histomorphological, clinical, and demographic data of 43 patients diagnosed with Bullous pemphigoid were analyzed. Immunohistochemical assessment of C4d expression and staining patterns was conducted. The findings suggest that C4d may serve as a potential diagnostic and prognostic marker. C4d expression was significantly more frequent in lesions located on the upper extremities and in patients with higher eosinophil scores. Furthermore, patients with high eosinophil scores were more likely to receive systemic treatment. This study demonstrates that C4d could contribute significantly to the diagnostic process, particularly in cases where immunofluorescence studies are limited.

摘要

炎症过程在自身免疫性和非自身免疫性皮肤病中均起着关键作用。补体系统参与这些过程,既有已明确的作用,也有尚待证实的影响。补体系统的中间产物C4d与皮肤病之间的关系仍是一个正在研究的领域。虽然已使用免疫组织化学方法探讨了C4d在自身免疫性和非自身免疫性皮肤病中的存在及作用,但该主题仍无定论。大疱性类天疱疮是一种自身免疫性皮肤病,其特征为表皮下水疱形成,主要影响老年人群。本研究旨在评估C4d作为金标准诊断方法——直接免疫荧光法的替代免疫组织化学标志物在诊断大疱性类天疱疮中的潜力。此外,分析C4d染色模式并与临床数据相关联,以评估其预后意义。在这项回顾性研究中,分析了43例诊断为大疱性类天疱疮患者的组织形态学、临床和人口统计学数据。进行了C4d表达和染色模式的免疫组织化学评估。研究结果表明,C4d可能作为一种潜在的诊断和预后标志物。C4d表达在上肢病变患者和嗜酸性粒细胞评分较高的患者中明显更常见。此外,嗜酸性粒细胞评分高的患者更有可能接受全身治疗。本研究表明,C4d可对诊断过程做出重大贡献,尤其是在免疫荧光研究受限的情况下。

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