Department of Dermatology, Ghent University Hospital, De Pintelaan 185, 9000, Ghent, Belgium.
Am J Clin Dermatol. 2017 Dec;18(6):733-744. doi: 10.1007/s40257-017-0298-5.
The pathophysiology of vitiligo is becoming increasingly clarified. In non-segmental vitiligo, early factors include activation of innate immunity, inflammasome activation, oxidative stress, and loss of melanocyte adhesion. Nonetheless, the main mechanism leading to non-segmental vitiligo involves an immune-mediated destruction of melanocytes. Anti-melanocyte-specific cytotoxic T cells exert a central role in the final effector stage. Genetic research revealed a multi-genetic inheritance displaying an overlap with other autoimmune disorders. However, some melanocyte-specific genes were also affected. Segmental vitiligo carries a different pathogenesis with most evidence indicating a mosaic skin disorder. Current management includes topical corticosteroids and immunomodulators. Narrow-band ultraviolet B can be used in patients not responding to topical treatment or in patients with extensive disease. Pigment cell transplantation offers an alternative for the treatment of segmental vitiligo or stable non-segmental lesions. Recent findings have revealed new targets for treatment that could lead to more efficient therapies. Targeted immunotherapy may halt the active immune pathways, although combination therapy may still be required to induce satisfying repigmentation. A recently established core set of outcome measures, new measurement instruments, and biomarker research pave the way for future standardized clinical trials.
白癜风的病理生理学机制日益明晰。在非节段性白癜风中，早期因素包括固有免疫激活、炎症小体激活、氧化应激和黑素细胞黏附丧失。尽管如此，导致非节段性白癜风的主要机制涉及免疫介导的黑素细胞破坏。抗黑素细胞特异性细胞毒性 T 细胞在终末效应阶段发挥核心作用。遗传研究揭示了一种多基因遗传，与其他自身免疫性疾病存在重叠。然而，一些黑素细胞特异性基因也受到影响。节段性白癜风具有不同的发病机制，大多数证据表明其为马赛克皮肤疾病。目前的治疗方法包括局部皮质类固醇和免疫调节剂。窄谱中波紫外线可用于对局部治疗无反应的患者或广泛疾病的患者。色素细胞移植为节段性白癜风或稳定的非节段性病变的治疗提供了一种替代方法。最近的发现揭示了新的治疗靶点，可能会带来更有效的治疗方法。靶向免疫疗法可能会阻断活跃的免疫途径，尽管可能仍需要联合治疗来诱导令人满意的复色。最近建立的一套核心疗效指标、新的测量仪器和生物标志物研究为未来的标准化临床试验铺平了道路。
