A comprehensive review of immune-mediated dermatopathology in systemic lupus erythematosus

J Autoimmun. 2018 Sep:93:1-15. doi: 10.1016/j.jaut.2018.07.007. Epub 2018 Jul 11.

Abstract

Lupus erythematosus (LE) is an autoimmune disease with a broad clinical spectrum ranging from cutaneous lesions to severe systemic manifestations. The pathogenesis of the disease and the immunological mechanisms for the heterogeneities in lupus remain unclear. The LE-specific cutaneous manifestations are generally divided into three categories: acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) and chronic cutaneous lupus erythematosus (CCLE). Clinically, lupus patients with skin lesions can be divided into two subsets based on the organs involved: cutaneous LE, such as DLE and SCLE, which appears only as a skin manifestation, and systemic lupus erythematosus (SLE), e.g., ACLE, which involves other organs, such as kidneys, joints, and the hematopoietic system. However, lupus is an aggressive disease, and cutaneous lupus and systemic lupus partially overlap. Fewer than 5% of DLE patients and approximately 50% of SCLE patients might develop major organ damage and then develop SLE in subsequent years. Furthermore, there are no predictive biomarkers in clinical use. To the best of our knowledge, increasing evidence from clinical trials has revealed that early intervention can either reduce or delay the onset of severe manifestations. Therefore, identification of certain biomarkers in skin lesions or circulation from patients with skin lesions to predict future flares and advise treatment is an unmet need. In this review, we comprehensively describe the subtypes of LE with pathological criteria and clinical manifestations; summarize the up-to-date evidence on certain cell distributions, such as keratinocytes, neutrophils, dendritic cells, T cells and B cells, in skin and peripheral blood; and discuss their pathogenic roles and their potential roles in predictive diagnosis and as therapeutic targets.

Keywords: B cells; DLE; Dendritic cells; SCLE; SLE; T cells.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Antibodies, Monoclonal / therapeutic use
  • B-Lymphocytes / drug effects
  • B-Lymphocytes / immunology
  • B-Lymphocytes / pathology*
  • Clinical Trials as Topic
  • Dendritic Cells / drug effects
  • Dendritic Cells / immunology
  • Dendritic Cells / pathology
  • Gene Expression Regulation
  • Humans
  • Interferon Regulatory Factors / genetics
  • Interferon Regulatory Factors / immunology
  • Joints / drug effects
  • Joints / immunology
  • Joints / pathology
  • Keratinocytes / drug effects
  • Keratinocytes / immunology
  • Keratinocytes / pathology*
  • Kidney / drug effects
  • Kidney / immunology
  • Kidney / pathology
  • Lupus Erythematosus, Discoid / genetics
  • Lupus Erythematosus, Discoid / immunology
  • Lupus Erythematosus, Discoid / pathology*
  • Lupus Erythematosus, Discoid / therapy
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology
  • Lupus Erythematosus, Systemic / pathology*
  • Lupus Erythematosus, Systemic / therapy
  • STAT4 Transcription Factor / genetics
  • STAT4 Transcription Factor / immunology
  • Skin / drug effects
  • Skin / immunology
  • Skin / pathology*
  • T-Lymphocytes / drug effects
  • T-Lymphocytes / immunology
  • T-Lymphocytes / pathology*
  • Toll-Like Receptors / genetics
  • Toll-Like Receptors / immunology

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Interferon Regulatory Factors
  • STAT4 Transcription Factor
  • STAT4 protein, human
  • Toll-Like Receptors