Dermatological Department, Istituto Dermopatico dell'Immacolata (IDI)-Scientific Institute for Research, Hospitalization and Healthcare (IRCCS), Rome, Italy.
Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata (IDI)-Scientific Institute for Research, Hospitalization and Healthcare (IRCCS), Rome, Italy.
Front Immunol. 2024 Aug 29;15:1416473. doi: 10.3389/fimmu.2024.1416473. eCollection 2024.
Bullous pemphigoid (BP) is the most common autoimmune bullous disease: it most commonly affects individuals over 70 years old and impacts severely on their quality of life. BP represents a paradigm for an organ-specific autoimmune disease and is characterized by circulating IgG autoantibodies to hemidesmosomal components: BP180 and BP230. While the crucial role of these autoantibodies in triggering BP inflammatory cascade is fully acknowledged, many ancillary etiological mechanisms need to be elucidated yet. Cutaneous melanoma is due to a malignant transformation of skin melanocytes, that produce and distribute pigments to surrounding keratinocytes. Melanoma is the most fatal skin cancer because of its increasing incidence and its propensity to metastasize. Several data such as: i) reported cases of concomitant melanoma and BP; ii) results from association studies; iii) BP onset following immune check-point inhibitors therapy; iv) expression of BP antigens in transformed melanocytes; and vi) circulating autoantibodies to BP antigens in melanoma patients suggest an intriguing, although unproven, possible association between melanoma and BP. However, a possible causative link is still debated and the putative pathogenetic mechanism underlying this association is unclear. This review aims to describe and discuss the possible relationship between BP and melanoma and give an overview of the speculations for or against this association. Of note, if demonstrated, this association could unwrap considerations of clinical relevance that represent new research frontiers.
大疱性类天疱疮（BP）是最常见的自身免疫性大疱性疾病：它最常影响 70 岁以上的个体，并严重影响他们的生活质量。BP 代表了一种器官特异性自身免疫性疾病的范例，其特征是存在针对半桥粒成分的循环 IgG 自身抗体：BP180 和 BP230。虽然这些自身抗体在引发 BP 炎症级联反应中的关键作用已得到充分认可，但仍需要阐明许多辅助病因机制。皮肤黑色素瘤是由于皮肤黑素细胞的恶性转化引起的，黑素细胞产生并分布色素到周围角质形成细胞。黑色素瘤是最致命的皮肤癌，因为其发病率不断增加且易于转移。有几项数据表明：i）同时患有黑色素瘤和 BP 的报告病例；ii）关联研究的结果；iii）免疫检查点抑制剂治疗后出现 BP 发作；iv）转化黑素细胞中 BP 抗原的表达；以及 v）黑色素瘤患者中针对 BP 抗原的循环自身抗体，提示黑色素瘤和 BP 之间存在有趣的、尽管未经证实的可能关联。然而，这种关联的潜在因果关系仍存在争议，其潜在的发病机制尚不清楚。本综述旨在描述和讨论 BP 与黑色素瘤之间可能存在的关系，并概述了对这种关联的正反两面的推测。值得注意的是，如果得到证实，这种关联可能会带来具有临床相关性的考虑，代表新的研究前沿。
