Lin Xiran, Meng Xianmin, Lin Jingrong
Department of Dermatology, First Affiliated Hospital of Dalian Medical University, Dalian, China.
Department of Pathology and Laboratory Medicine, Axia Women's Health, Oaks, PA, USA.
Int J Dermatol. 2025 Mar;64(3):490-498. doi: 10.1111/ijd.17627. Epub 2024 Dec 30.
Vitiligo is a common depigmentation disorder classified into nonsegmental vitiligo (NSV) and segmental vitiligo (SV). SV accounts for 5-27.9% of patients with vitiligo. The primary pathogenesis of NSV involves the autoimmune-mediated destruction of melanocytes. Recently, an autoimmune pathogenesis of SV was identified. High levels of melanocyte antigen-specific CD8+ T cells are found in early SV lesional skin infiltrating around melanocytes along the basal layer. Mixed vitiligo suggests an overlap in pathogenesis between SV and NSV. In active SV, serum innate immune cytokines, and CD8+ T cell cytokines are increased. Oxidative stress in SV may activate autoimmune responses. SV pathogenesis is associated with a local cytotoxic response targeting epidermal melanocytes. Theories have been put forward to explain the segmental pattern in SV. The previous basis of the neurogenic theory that SV results from dermatomes is no longer accepted. However, there are still research reports supporting this theory. Evaluating the distribution pattern of SV lesions has provided clues to the mosaicism detection of suspected melanocytic defects at the site of SV lesions, supporting this theory. Evidence points to a cytotoxic response targeting mosaic melanocytes. Understanding SV's autoimmune pathogenesis prompts a reevaluation of immunosuppressive medical treatments for SV. The excellent results of autologous melanocyte transplantation in SV lesions compared with the moderate to limited results in patients with NSV support the mosaicism theory.
白癜风是一种常见的色素脱失性疾病，分为非节段型白癜风（NSV）和节段型白癜风（SV）。SV占白癜风患者的5%-27.9%。NSV的主要发病机制涉及自身免疫介导的黑素细胞破坏。最近，SV的自身免疫发病机制被确定。在早期SV皮损中，沿着基底层在黑素细胞周围浸润的皮肤中发现高水平的黑素细胞抗原特异性CD8+T细胞。混合型白癜风提示SV和NSV在发病机制上存在重叠。在活动期SV中，血清固有免疫细胞因子和CD8+T细胞因子增加。SV中的氧化应激可能激活自身免疫反应。SV的发病机制与针对表皮黑素细胞的局部细胞毒性反应有关。已经提出了一些理论来解释SV中的节段模式。以前认为SV由皮节引起的神经源性理论已不再被接受。然而，仍有研究报告支持这一理论。评估SV皮损的分布模式为检测SV皮损部位疑似黑素细胞缺陷的镶嵌现象提供了线索，支持了这一理论。有证据表明存在针对镶嵌黑素细胞的细胞毒性反应。了解SV的自身免疫发病机制促使人们重新评估SV的免疫抑制药物治疗。与NSV患者中中度至有限的结果相比，自体黑素细胞移植在SV皮损中的优异结果支持了镶嵌现象理论。
