title: Lupus erythematosus revisited.
abstract: Lupus erythematosus (LE) is a multifactorial autoimmune disease with clinical manifestations of differing severity. The exact pathomechanisms and interactions resulting in the inflammatory and immunological processes of this heterogeneous disease remain elusive. Approaches in the understanding of the pathomechanisms revealed that the clinical expression of LE is predisposed by susceptibility genes and that various environmental factors are responsible for an abnormal immune response. Several studies demonstrated that ultraviolet (UV) light is one of the major factors in the pathogenesis of the disease. Standardized photoprovocation in patients with LE has been shown to be a safe and efficient model for evaluating the underlying pathomechanisms which lead to the production of autoantibodies and immune complexes. In particular, interferons were defined as important players in the early activation of the immune system and were observed to play a specific role in the immunological interface between the innate and the adaptive immune system. Abnormalities or disturbances in the different processes of cell death, such as apoptosis or necrosis, have also been recognized as crucial in the pathogenesis of LE. Although each process is different and characterized by unique features, the processes are interrelated and result in a complex disease.
authors.affiliation: Interdisciplinary Center for Clinical Trials (IZKS), University Medical Center Mainz, Langenbeckstrasse 1, 55131, Mainz, Germany. research@izks-unimedizin-mainz.de.|Division of Immunogenetics, Tumor Immunology Program, German Cancer Research Center (DKFZ), Heidelberg, Germany. research@izks-unimedizin-mainz.de.
authors.forename: Annegret
authors.initials: A
authors.lastname: Kuhn
authors.affiliation: Department of Dermatology, University Hospital Bonn, Bonn, Germany.
authors.forename: Joerg
authors.initials: J
authors.lastname: Wenzel
authors.affiliation: Department of Internal Medicine and Rheumatology, Martini Hospital, Groningen, Netherlands.
authors.forename: Marc
authors.initials: M
authors.lastname: Bijl
mesh_terms: D000818:Animals; D016923:Cell Death; D024243:HMGB1 Protein / Q000235:genetics / Q000378:metabolism; D006801:Humans; D007372:Interferons / Q000378:metabolism; D008178:Lupus Erythematosus, Cutaneous / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000209:etiology* / Q000378:metabolism; D010787:Photosensitivity Disorders / Q000150:complications; D012867:Skin / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D014466:Ultraviolet Rays / Q000009:adverse effects
chemical_list: D024243:HMGB1 Protein; D007372:Interferons
keywords: Apoptosis; Cutaneous lupus erythematosus; Interferons; Necrosis; Pathogenesis; Photoprovocation; Photosensitivity; Systemic lupus erythematosus; UV light