Chiewchengchol Direkrit, Murphy Ruth, Edwards Steven W, Beresford Michael W
Institute of Translational Medicine, Alder Hey Children's NHS Foundation Trust, University of Liverpool, Liverpool, UK ; Institute of Integrative Biology, University of Liverpool, Liverpool, UK ; Immunology Unit & Center of Excellence in Immunology and Immune-mediated Disease, Department of Microbiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Department of Dermatology, Queens Medical Centre, Nottingham University Teaching Hospitals, Nottingham, UK.
Pediatr Rheumatol Online J. 2015 Jan 5;13:1. doi: 10.1186/1546-0096-13-1. eCollection 2015.
Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) and those strongly associated with and forming part of the diagnostic spectrum, but without the classical histological changes of lupus (LE nonspecific). A malar rash is the most commonly associated LE specific dermatological presentation. This skin manifestation is an acute form and also correlates with disease activity. Subacute (polycyclic or papulosquamous lesions) and chronic (discoid lesions) forms, whilst showing classical histological changes supportive of lupus, are less commonly associated with systemic lupus and do not correlate with disease activity. The most commonly associated skin lesions without classical lupus changes are cutaneous vasculitis, oral ulcers and diffuse non-scarring alopecia. These signs frequently relate to disease activity. An understanding of cutaneous signs and symptoms of lupus in children is important to avoid delay in diagnosis. They will often improve as lupus is adequately controlled and their reappearance is often the first indicator of a disease flare.
被诊断为青少年型系统性红斑狼疮（JSLE）的患者在发病时常常伴有皮肤和口腔损害。这些黏膜皮肤损害，根据美国风湿病学会（ACR）1997年的定义，包括颧部红斑、盘状红斑、光过敏和口腔溃疡。因此，识别黏膜皮肤损害对于准确诊断JSLE至关重要。黏膜皮肤损害可分为具有典型组织学特征的（狼疮特异性）和与狼疮诊断范围密切相关且构成其一部分，但无狼疮典型组织学改变的（狼疮非特异性）。颧部红斑是最常见的狼疮特异性皮肤表现。这种皮肤表现为急性形式，也与疾病活动相关。亚急性（多环状或丘疹鳞屑性损害）和慢性（盘状损害）形式，虽然显示支持狼疮的典型组织学改变，但较少与系统性红斑狼疮相关，且与疾病活动无关。最常见的无狼疮典型改变的皮肤损害是皮肤血管炎、口腔溃疡和弥漫性非瘢痕性脱发。这些体征常与疾病活动相关。了解儿童狼疮的皮肤体征和症状对于避免诊断延误很重要。随着狼疮得到充分控制，这些症状通常会改善，而它们的再次出现往往是疾病复发的首要指标。
