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自身免疫性皮肤疾病:红斑狼疮。

Autoimmune Skin Conditions: Cutaneous Lupus Erythematosus.

机构信息

Department of Dermatology - Penn State College of Medicine, 700 HMC Crescent Road, Hershey, PA 17033.

Department of Family and Community Medicine - Penn State College of Medicine, 700 HMC Crescent Road, Hershey, PA 17033.

出版信息

FP Essent. 2023 Mar;526:25-36.

Abstract

Cutaneous lupus erythematosus (CLE) is a spectrum of autoimmune skin conditions associated with systemic lupus erythematosus (SLE). CLE and SLE may exist concurrently or independently. Accurate recognition of CLE is crucial because it may herald systemic disease onset. Lupus-specific skin conditions include acute cutaneous lupus erythematosus (ACLE) which manifests as a malar or butterfly rash; subacute cutaneous lupus erythematosus (SCLE); and chronic cutaneous lupus erythematosus, which includes discoid lupus erythematosus (DLE). All three types of CLE present as pink-violet macules or plaques with unique morphology, in areas of sun-exposed skin. Association with SLE differs: ACLE is most closely associated, with SCLE in the middle, and DLE the least so. All types of CLE are pruritic, sting, and burn, and DLE can result in disfiguring scarring. All CLE is exacerbated by UV light exposure and smoking. Diagnosis combines clinical evaluation with skin biopsy. Management focuses on mitigating modifiable risk factors and using pharmacotherapy. UV protection includes use of sun protective factor (SPF) 60 or higher sunscreens containing zinc oxide or titanium dioxide, avoidance of sun exposure, and use of physical barrier clothing. Topical therapies and antimalarial drugs are first-line, followed by systemic therapies (eg, disease-modifying antirheumatic drugs, biologic therapies [eg, anifrolumab, belimumab], or other advanced systemic drugs).

摘要

皮肤狼疮(CLE)是与系统性红斑狼疮(SLE)相关的一系列自身免疫性皮肤疾病。CLE 和 SLE 可能同时存在或独立存在。准确识别 CLE 至关重要,因为它可能预示着系统性疾病的发作。狼疮特异性皮肤疾病包括急性皮肤狼疮(ACLE),表现为蝶形或蝴蝶状皮疹;亚急性皮肤狼疮(SCLE);以及慢性皮肤狼疮,包括盘状狼疮(DLE)。所有三种类型的 CLE 都表现为在暴露于阳光的皮肤区域出现粉红色-紫色斑疹或斑块,具有独特的形态。与 SLE 的关联不同:ACLE 最为密切相关,SCLE 居中,DLE 则不太相关。所有类型的 CLE 都伴有瘙痒、刺痛和烧灼感,DLE 可导致毁容性瘢痕。所有 CLE 都会因紫外线照射和吸烟而加重。诊断结合临床评估和皮肤活检。治疗重点在于减轻可改变的危险因素,并使用药物治疗。紫外线防护包括使用防晒系数(SPF)为 60 或更高的防晒霜,其中含有氧化锌或二氧化钛,避免阳光照射,并使用物理屏障服装。局部治疗和抗疟药物是一线治疗,其次是系统治疗(例如,疾病修饰抗风湿药物、生物疗法[例如,anifrolumab、belimumab]或其他先进的系统药物)。

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