Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature.
Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature.
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DOI：
10.1186/1546-0096-13-1
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
Beresford MW
中科院分区：
文献类型：
--
作者：
Chiewchengchol D;Murphy R;Edwards SW;Beresford MW
被诊断为青少年起病的系统性红斑狼疮(JSLE)的患者通常有皮肤和口腔损害作为其表现的一部分。根据美国风湿病学会(ACR)1997年的定义，这些黏膜皮肤损害包括颧部皮疹、盘状皮疹、光敏和口腔溃疡。因此，识别皮肤粘膜病变对于准确诊断JSLE是至关重要的。皮肤粘膜损害可分为典型的组织学特征(LE特异性)和与狼疮的典型组织学改变(LE非特异性)无典型组织学改变(LE非特异性)的皮肤黏膜损害。颧部皮疹是最常见的LE特异性皮肤病表现。这种皮肤表现是一种急性形式，也与疾病活动有关。亚急性(多环或丘疹鳞状病变)和慢性(盘状病变)，虽然表现出支持狼疮的经典组织学变化，但较少与系统性狼疮相关，且与疾病活动性无关。最常见的没有典型狼疮改变的皮肤病变是皮肤血管炎、口腔溃疡和弥漫性无疤痕脱发。这些体征通常与疾病活动有关。了解儿童狼疮的皮肤症状和体征对于避免延误诊断非常重要。当狼疮得到充分控制时，它们通常会改善，而且它们的再次出现往往是疾病发作的第一个指标。
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.
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