【皮肤型红斑狼疮的诊治】
作者：Emily Z 翻译：柴静（chaijing1008@163.com） 校正：赵金霞 发布者：金银姬 校对：武东
摘要
皮肤型红斑狼疮的皮肤表现多种多样，可能与系统性疾病有关，也可能无关。该领域近期的研究让我们对该病的理解及其治疗选择更加清晰明了。流行病学研究发现皮肤型红斑狼疮的发病率为4.3/100000，基本接近系统性红斑狼疮的相关分析。尽管为了定义系统性红斑狼疮做了大量的工作，皮肤型红斑狼疮及其亚型分类依然是个挑战。目前诊断主要依靠临床和实验室发现以及皮肤组织学。皮肤红斑区和严重性指数™ (CLASI™)是疾病活动度及损伤程度的可靠手段。皮肤型红斑狼疮的发病由多种因素参与，包括遗传因素及紫外线作用，也与免疫紊乱和参与细胞因子瀑布的异常信号通路有关。患者教育和避免触发因素是疾病预防的关键，抗疟药和局部应用激素依然是标准治疗，但是，免疫抑制剂、沙利度胺类似物和单克隆抗体可能作为系统性疗法治疗难治型皮肤型狼疮。
附原文：
Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic disease. Recent studies in this area continue to shape our understanding of this disease and treatment options. Epidemiologic studies have found an incidence of CLE of 4.30 per 100,000, which approaches similar analysis for systemic lupus erythematosus (SLE). Although there have been extensive efforts to define SLE, the classification of CLE and its subgroups remains a challenge. Currently, diagnosis relies on clinical and laboratory findings as well as skin histology. The Cutaneous Lupus Area and Severity Index™ (CLASI™) is a validated measure of disease activity and damage. CLE pathogenesis is multifactorial and includes genetic contributions as well as effects of ultraviolet (UV) light. Immune dysregulation and aberrant cell signaling pathways through cytokine cascades are also implicated. Patient education and avoidance of triggers are key to disease prevention. Antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for the treatment of recalcitrant disease.
引自：Emily Z. Hejazi; Victoria P. Werth Cutaneous Lupus Erythematosus: An Update on Pathogenesis, Diagnosis and Treatment.Am J Clin Dermatol. 2016;17(2):135-146.
摘要
皮肤型红斑狼疮的皮肤表现多种多样，可能与系统性疾病有关，也可能无关。该领域近期的研究让我们对该病的理解及其治疗选择更加清晰明了。流行病学研究发现皮肤型红斑狼疮的发病率为4.3/100000，基本接近系统性红斑狼疮的相关分析。尽管为了定义系统性红斑狼疮做了大量的工作，皮肤型红斑狼疮及其亚型分类依然是个挑战。目前诊断主要依靠临床和实验室发现以及皮肤组织学。皮肤红斑区和严重性指数™ (CLASI™)是疾病活动度及损伤程度的可靠手段。皮肤型红斑狼疮的发病由多种因素参与，包括遗传因素及紫外线作用，也与免疫紊乱和参与细胞因子瀑布的异常信号通路有关。患者教育和避免触发因素是疾病预防的关键，抗疟药和局部应用激素依然是标准治疗，但是，免疫抑制剂、沙利度胺类似物和单克隆抗体可能作为系统性疗法治疗难治型皮肤型狼疮。
附原文：
Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic disease. Recent studies in this area continue to shape our understanding of this disease and treatment options. Epidemiologic studies have found an incidence of CLE of 4.30 per 100,000, which approaches similar analysis for systemic lupus erythematosus (SLE). Although there have been extensive efforts to define SLE, the classification of CLE and its subgroups remains a challenge. Currently, diagnosis relies on clinical and laboratory findings as well as skin histology. The Cutaneous Lupus Area and Severity Index™ (CLASI™) is a validated measure of disease activity and damage. CLE pathogenesis is multifactorial and includes genetic contributions as well as effects of ultraviolet (UV) light. Immune dysregulation and aberrant cell signaling pathways through cytokine cascades are also implicated. Patient education and avoidance of triggers are key to disease prevention. Antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for the treatment of recalcitrant disease.
引自：Emily Z. Hejazi; Victoria P. Werth Cutaneous Lupus Erythematosus: An Update on Pathogenesis, Diagnosis and Treatment.Am J Clin Dermatol. 2016;17(2):135-146.
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