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[红斑狼疮。通过临床变异、相关疾病及模仿者呈现出广泛的症状]

[Lupus erythematosus. Wide range of symptoms through clinical variation, associated diseases and imitators].

作者信息

Aberer E

机构信息

Univ.-Klinik für Dermatologie, Medizinische Universität Graz, Auenbrugger Platz 8, 8036, Graz, Osterreich.

出版信息

Hautarzt. 2010 Aug;61(8):676-82. doi: 10.1007/s00105-010-1939-2.

Abstract

The typical clinical forms of cutaneous lupus erythematosus (LE) are the butterfly rash, acute, subacute and chronic cutaneous lupus, intermediate lupus (lupus tumidus), chilblain- and bullous lupus, lupus profundus, and ulcerating lesions on the mucous membrane. Besides the typical lupus forms, nonspecific skin lesions are also observed such as dermal mucinosis, acneiform skin lesions, different variants of livedo, necrotizing vasculitis with ulcers, purpura, urticaria vasculitis, neutrophilic dermatosis, hyperpigmentation, hair and nail changes as well as overlap syndromes with erythema multiforme, scleroderma, Sjögren syndrome, Raynaud phenomenon, lichen planus, bullous pemphigoid und psoriasis. There are lupus imitators which create differential diagnostic challenges, such as infections with atypical mycobacteria or subcutaneous T-cell lymphoma both of which are similar to lupus profundus. All these skin lesions can present as maximal pathological findings seen in lupus or be caused by a variety of pathological laboratory findings such as the anti-phospholipid antibodies or a deficiency of complement factors. In the latter situation severe lupus often with complications can be expected.

摘要

皮肤红斑狼疮(LE)的典型临床形式包括蝶形红斑、急性、亚急性和慢性皮肤型红斑狼疮、中间型红斑狼疮(肿胀性红斑狼疮)、冻疮样和大疱性红斑狼疮、深部红斑狼疮以及黏膜溃疡病变。除了典型的红斑狼疮形式外,还观察到非特异性皮肤病变,如皮肤黏蛋白沉积症、痤疮样皮肤病变、不同类型的青斑、伴有溃疡的坏死性血管炎、紫癜、荨麻疹性血管炎、嗜中性皮病、色素沉着、毛发和指甲变化,以及与多形红斑、硬皮病、干燥综合征、雷诺现象、扁平苔藓、大疱性类天疱疮和银屑病的重叠综合征。存在一些会带来鉴别诊断挑战的红斑狼疮模仿者,如非典型分枝杆菌感染或皮下T细胞淋巴瘤,这两者都与深部红斑狼疮相似。所有这些皮肤病变都可能表现为红斑狼疮中所见的最大病理发现,或者由各种病理实验室检查结果引起,如抗磷脂抗体或补体因子缺乏。在后一种情况下,通常会出现伴有并发症的重症红斑狼疮。

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