Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

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• 作者：Direkrit Chiewchengchol (1) (2) (3)
  Ruth Murphy (4)
  Steven W Edwards (2)
  Michael W Beresford (1) (5)
  
  1. Institute of Translational Medicine ; Alder Hey Children鈥檚 NHS Foundation Trust ; University of Liverpool ; Liverpool ; UK
  2. Institute of Integrative Biology ; University of Liverpool ; Liverpool ; UK
  3. Immunology Unit & Center of Excellence in Immunology and Immune-mediated Disease ; Department of Microbiology ; Faculty of Medicine ; Chulalongkorn University ; Bangkok ; Thailand
  4. Department of Dermatology ; Queens Medical Centre ; Nottingham University Teaching Hospitals ; Nottingham ; UK
  5. Department of Women鈥檚 and Children鈥檚 Health ; Institute of Translational Medicine ; Alder Hey Children鈥檚 NHS Foundation Trust ; Eaton Road ; Liverpool ; L12 2AP ; UK
  
• 关键词：Juvenile ; onset systemic lupus erythematosus ; Mucocutaneous lupus lesions ; Lupus erythematosus specific lesions ; Lupus erythematosus nonspecific lesions ; Diagnosis ; Treatment
• 刊名：Pediatric Rheumatology
• 出版年：2015
• 出版时间：December 2015
• 年：2015
• 卷：13
• 期：1
• 全文大小：825 KB
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• 刊物主题：Pediatrics; Rheumatology;
• 出版者：BioMed Central
• ISSN：1546-0096

文摘

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.