Abstract
Cutaneous lupus erythematosus can be a devastating painful and mutilating disease that is associated with an inflammatory response in the skin driven by type I interferon activation. Clearance defects in the extra- and intracellular space lead to an enhanced prevalence of nucleic acids that represent danger signals for the innate immune system. Self nucleic acids can stimulate DNA and RNA sensors that have originally evolved to ensure viral defense. Their activation can induce a type I interferon dominated response in resident skin cells, macrophages and dendritic cells that subsequently progresses to adaptive immune stimulation. The genetic exploration of rare monogenic type I interferon driven diseases helped to identify these pathogenic concepts. Based on a genetic susceptibility lupus patients are more vulnerable to environmental trigger factors such as UV-irradiation that can provoke inflammation with local tissue destruction and eventually systemic disease. Understanding of these pathogenic concepts is a prerequisite for development of targeted therapies.
Highlights
Lupus erythematosus is a chronic relapsing autoimmune disease with a varying spectrum of clinical manifestations ranging from sole cutaneous involvement to fatal multi-organ disease [1]
Immune complexes containing autoantibodies to nucleic acid binding proteins and nucleic acids are frequently deposited along the basement membrane zone of the skin and the concentration of autoantibodies against double stranded DNA correlates with systemic involvement and disease activity
This review aims to provide an overview on the role of innate immune processes caused by disturbances in the normal restriction and regulation of the nucleic acid metabolism in the pathogenesis of cutaneous lupus erythematosus
Summary
Self nucleic acids can stimulate DNA and RNA sensors that have originally evolved to ensure viral defense. Their activation can induce a type I interferon dominated response in resident skin cells, macrophages and dendritic cells that subsequently progresses to adaptive immune stimulation. Based on a genetic susceptibility lupus patients are more vulnerable to environmental trigger factors such as UV-irradiation that can provoke inflammation with local tissue destruction and eventually systemic disease. Understanding of these pathogenic concepts is a prerequisite for development of targeted therapies
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