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红斑狼疮:皮肤科发现的意义。

Lupus erythematosus: Significance of dermatologic findings.

机构信息

Faculty of medicine, University of Strasbourg, and Dermatology clinic, Strasbourg university hospital, Strasbourg, France.

Faculty of medicine, University of Strasbourg, and Dermatology clinic, Strasbourg university hospital, Strasbourg, France.

出版信息

Ann Dermatol Venereol. 2021 Mar;148(1):6-15. doi: 10.1016/j.annder.2020.08.052. Epub 2021 Jan 19.

Abstract

Herein, the different skin manifestations in patients with lupus erythematosus are reviewed, and their diagnostic, pathogenic and prognostic relevance are discussed, as well as their impact on therapeutic choices. The so-called specific lesions of LE result from an autoimmune pathomechanism and they allow diagnosis of LE by simple clinicopathological correlation since the findings are characteristic. They include the classic acute, subacute and chronic variants, characterised microscopically by interface dermatitis; the dermal variants of lupus, such as tumid lupus, displaying dermal perivascular lymphocytic infiltrate with mucin deposition under the microscope, and lupus profundus, in which lymphocytic lobular panniculitis progressing to hyaline fibrosis is found. Antimalarials are the treatment of choice for patients with specific LE lesions. The presence of some dermatological signs is the result of thrombotic vasculopathy. Their recognition allows the identification of lupus patients at increased cardiovascular risk and with a worse overall prognosis. Those signs include reticulated erythema on the tip of the toes, splinter hemorrhages, atrophie blanche, pseudo-Degos lesions, racemosa-type livedo, anetoderma, ulceration and necrosis. Those clinical manifestations, often subtle, must be recognised, and if present, patients should be treated with antiplatelet drugs. Finally, neutrophilic cutaneous lupus erythematosus includes a few entities that suggest that autoinflammatory mechanisms might play a key role in certain lupus manifestations. Among those entities, it is very important to diagnose neutrophilic urticarial dermatosis, which can mimic a classic lupus flare, because it is characterised by rash with joint pain, but immunosuppressants are not helpful. Dapsone is the treatment of choice.

摘要

本文回顾了红斑狼疮患者的不同皮肤表现,并讨论了它们的诊断、发病机制和预后意义,以及它们对治疗选择的影响。所谓的 LE 特异性病变是由自身免疫发病机制引起的,通过简单的临床病理相关性即可诊断 LE,因为其表现具有特征性。它们包括经典的急性、亚急性和慢性变体,显微镜下表现为界面性皮炎;狼疮的皮肤变体,如肿胀性狼疮,表现为真皮血管周围淋巴细胞浸润,伴有显微镜下粘蛋白沉积;狼疮深部,表现为淋巴细胞小叶性脂膜炎进展为透明性纤维化。对于有特异性 LE 病变的患者,抗疟药是首选治疗方法。某些皮肤迹象的出现是血栓血管病变的结果。识别它们可以识别出心血管风险增加和整体预后较差的狼疮患者。这些迹象包括脚趾尖端的网状红斑、裂片状出血、苍白萎缩、假性 Degos 病变、racemosa 型网状青斑、网状皮肤萎缩、溃疡和坏死。这些临床表现常常很微妙,必须被识别,如果存在,应使用抗血小板药物进行治疗。最后,中性粒细胞性皮肤红斑狼疮包括一些实体,表明自身炎症机制可能在某些狼疮表现中起关键作用。在这些实体中,诊断中性粒细胞性荨麻疹样皮肤病非常重要,因为它可能模仿典型的狼疮发作,其特征是皮疹伴关节痛,但免疫抑制剂没有帮助。氨苯砜是首选治疗方法。

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