Philadelphia VA Medical Center, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.
Curr Opin Rheumatol. 2013 Sep;25(5):584-90. doi: 10.1097/BOR.0b013e32836437ba.
Cutaneous Lupus Erythematous (CLE) is an autoimmune disease in which patients may present with isolated skin findings or have CLE associated with underlying systemic disease. The most significant recent studies on its pathogenesis and therapeutic management are reviewed here.
Patients with subacute and Discoid Lupus Erythematous had elevated Interferon score, about a third of all cases of SCLE could be attributed to previous drug exposure, and smoking may be more closely associated with CLE than Systemic Lupus Erythematous (SLE). An underlying genetic defect in some subsets of CLE patients may also be shared with SLE. Efficacy of antimalarial therapy is enhanced by increasing treatment duration or maintaining higher blood drug concentrations. Combination antimalarials that include quinacrine, thalidomide analogs, and Mycophenalate Mofetil may also be effective in refractory CLE.
The pathogenesis of CLE remains unclear, and is likely multifactorial. Identified associations with subsets of CLE suggest future research questions in CLE pathogenesis. Subsets of CLE associated with interface dermatitis may share an underlying genetic defect in interferon signaling with SLE. The Cutaneous Lupus Disease Area and Severity Index is a valuable and widely used tool allowing standardized assessment and reporting of cutaneous disease activity and damage. More evidence is available to guide treatment of refractory CLE, but larger studies are needed.
红斑狼疮（CLE）是一种自身免疫性疾病，患者可能仅表现为皮肤病变，也可能与潜在的系统性疾病有关。本文对其发病机制和治疗管理方面的最新重要研究进行了回顾。
亚急性和盘状红斑狼疮患者的干扰素评分升高，大约三分之一的 SCLE 病例可归因于先前的药物暴露，吸烟与 CLE 的相关性可能比系统性红斑狼疮（SLE）更为密切。CLE 患者的某些亚群中也可能存在与 SLE 共享的潜在遗传缺陷。延长抗疟药物治疗时间或维持较高的血药浓度可提高其疗效。包括奎纳克林、沙利度胺类似物和霉酚酸酯在内的联合抗疟药物也可能对难治性 CLE 有效。
CLE 的发病机制仍不清楚，可能是多因素的。与 CLE 亚群的相关关联表明，CLE 发病机制的未来研究问题。与界面性皮炎相关的 CLE 亚群可能与 SLE 一样存在干扰素信号转导的潜在遗传缺陷。皮肤狼疮疾病面积和严重程度指数是一种有价值且广泛使用的工具，可用于标准化评估和报告皮肤疾病活动和损伤。目前有更多的证据可用于指导难治性 CLE 的治疗，但仍需要更大规模的研究。
