Department of Dermatology and Allergy, University Hospital of Bonn, Bonn, Germany.
Nat Rev Rheumatol. 2019 Sep;15(9):519-532. doi: 10.1038/s41584-019-0272-0. Epub 2019 Aug 9.
Cutaneous lupus erythematosus (CLE) is an autoimmune disease that can present as an isolated skin disease or as a manifestation within the spectrum of systemic lupus erythematosus. The clinical spectrum of CLE is broad, ranging from isolated discoid plaques to widespread skin lesions. Histologically, skin lesions present as interface dermatitis (inflammation of the skin mediated by anti-epidermal responses), which is orchestrated by type I and type III interferon-regulated cytokines and chemokines. Both innate and adaptive immune pathways are strongly activated in the formation of skin lesions owing to continuous re-activation of innate pathways via pattern recognition receptors (PRRs). These insights into the molecular pathogenesis of skin lesions in CLE have improved our understanding of the mechanisms underlying established therapies and have triggered the development of targeted treatment strategies that focus on immune cells (for example, B cells, T cells or plasmacytoid dendritic cells), as well as immune response pathways (for example, PRR signalling, Janus kinase (JAK)-signal transducer and activator of transcription (STAT) signalling and nuclear factor-κB signalling) and their cytokines and chemokines (for example, type I interferons, CXC-chemokine ligand 10 (CXCL10), IL-6 and IL-12).
皮肤狼疮红斑（CLE）是一种自身免疫性疾病，可以表现为孤立性皮肤疾病或系统性红斑狼疮的表现之一。CLE 的临床谱很广泛，从孤立性盘状斑块到广泛的皮肤损伤。组织学上，皮肤损伤表现为界面性皮炎（由针对表皮的反应介导的皮肤炎症），这是由 I 型和 III 型干扰素调节的细胞因子和趋化因子协调的。由于先天途径通过模式识别受体（PRR）持续被重新激活，固有和适应性免疫途径在皮肤损伤的形成中被强烈激活。这些对 CLE 皮肤损伤分子发病机制的认识提高了我们对既定治疗机制的理解，并引发了靶向治疗策略的发展，这些策略侧重于免疫细胞（例如 B 细胞、T 细胞或浆细胞样树突状细胞）以及免疫反应途径（例如 PRR 信号、Janus 激酶（JAK）-信号转导和转录激活因子（STAT）信号和核因子-κB 信号）及其细胞因子和趋化因子（例如 I 型干扰素、CXC 趋化因子配体 10（CXCL10）、IL-6 和 IL-12）。
