Intrinsic and extrinsic pathomechanisms in vitiligo
Intrinsic and extrinsic pathomechanisms in vitiligo
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DOI：
10.1034/j.1600-0749.13.s8.9.x
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发表时间：
2000-01-01
期刊：
影响因子：
--
通讯作者：
Taïeb, A
中科院分区：
文献类型：
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作者：
Taïeb, A
白癜风是最常见的获得性黑色素减少症，局限于有限的皮肤区域（局灶性/节段性白癜风[SV]）或全身对称斑块（非节段性白癜风[NSV]）。在大多数情况下，白癜风对应于黑素细胞的损失，首先在表皮隔室中，然后在大多数黑素细胞干细胞可能位于的滤泡储库中。目前有许多数据支持表皮黑色素单位的氧化还原状态受损是导致NSV中不适当免疫应答的主要缺陷。SV可能是黑素细胞丢失的镶嵌性发育倾向，其皮肤分布和自体移植的成功表明，在有限的范围内具有相似的机制。在NSV中，自体黑素细胞的植入不太持久，特别是在易于重复创伤或压力的区域。虽然黑素细胞是该疾病的明显目标，但角质形成细胞作为黑素细胞的抗氧化分子的提供者以及黑素合成中的辅助因子，可能参与其中。自身抗体和特异性细胞毒性T细胞的产生在不稳定型寻常型白癜风中朗格汉斯细胞大量摄取黑素细胞抗原的背景下并不令人惊讶，从而允许病变的自我延续。本文根据临床分类和拟议病理机制的内在/外在性质，回顾了有关白癜风病理生理学的最新数据。不幸的是，病理分期、临床评分和诱发因素等基本问题尚未完全解决。还需要在白癜风易感家族和与白癜风相关的特定遗传疾病中进行更多的遗传研究。
Vitiligo is the most commonly acquired hypomelanosis, and is restricted to a limited cutaneous territory (focal/segmental vitiligo [SV]) or generalized in symmetric patches (nonsegmental vitiligo [NSV]). in the majority of cases, vitiligo corresponds to a loss of melanocytes, first in the epidermal compartment, and later in the follicular reservoir where most melanocytic stem cells are probably situated. There are many data currently supporting an impaired redox status of the epidermal melanin unit as a primary defect leading to inappropriate immune responses in NSV. SV is probably a mosaic developmental predisposition to melanocytic loss, with similar mechanisms at work on a limited scale, as suggested by its cutaneous distribution and success of autografting. In NSV, engraftment of autologous melanocytes is less durable, especially in areas prone to repeated trauma or pressure. Although melanocytes are the obvious target of the disease, keratinocytes, as providers of antioxidant molecules to melanocytes as well as cofactors in the synthesis of melanin, are probably involved. The production of autoantibodies and specific cytotoxic T cells is not surprising in the context of the massive uptake of melanocytic antigens by Langerhans cells in unstable vitiligo vulgaris, thereby allowing the self-perpetuation of lesions. This article reviews the recent data on the pathophysiology of vitiligo, on the basis of clinical classification and the intrinsic/extrinsic nature of proposed pathomechanisms, Unfortunately, basic issues like pathological staging, clinical scoring, and eliciting factors have not yet been fully resolved. More genetic studies in vitiligo-prone families and in specific genetic disorders associated with vitiligo are also needed.