title: Dermatomyositis.
abstract: Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Although thought to be autoimmune in origin, many questions remain as to the etiopathogenesis of this disease. DM has classically been considered a humorally mediated disease. Current evidence, however, seems to increasingly support alternative (though not mutually exclusive) mechanisms of pathogenesis, including cell-mediated and innate immune system dysfunction. Pathologic findings of DM in muscle include infarcts, perifascicular atrophy, endothelial cell swelling and necrosis, vessel wall membrane attack complex deposition, and myocyte-specific MHC I upregulation. As for the skin, histopathologic findings include hyperkeratosis, epidermal basal cell vacuolar degeneration and apoptosis, increased dermal mucin deposition, and a cell-poor interface dermatitis. Autoantibodies, particularly those that bind nuclear or cytoplasmic ribonucleoprotein antigens, are also commonly found in DM, although their importance in pathogenesis remains unclear. Defective cellular clearance, genetic predilection and environmental exposures, such as viral infection, may also play an important role in the pathogenesis of DM. The seminal work regarding the pathogenesis of DM is reviewed and an update on the recent basic and molecular advances in the field is provided.
authors.affiliation: Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA 19119, USA.
authors.forename: M S
authors.initials: MS
authors.lastname: Krathen
authors.forename: D
authors.initials: D
authors.lastname: Fiorentino
authors.forename: V P
authors.initials: VP
authors.lastname: Werth
mesh_terms: D000818:Animals; D000974:Antibodies, Antinuclear / Q000276:immunology; D000917:Antibody Formation / Q000235:genetics; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000209:etiology / Q000235:genetics / Q000276:immunology / Q000473:pathology; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D002908:Chronic Disease; D003882:Dermatomyositis / Q000209:etiology / Q000235:genetics / Q000276:immunology / Q000473:pathology; D004730:Endothelium, Vascular / Q000276:immunology / Q000473:pathology; D015395:Histocompatibility Antigens Class I / Q000235:genetics / Q000276:immunology; D006801:Humans; D007111:Immunity, Cellular / Q000235:genetics; D007113:Immunity, Innate; D032342:Muscle Cells / Q000276:immunology / Q000473:pathology; D009132:Muscles / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology; D015854:Up-Regulation / Q000235:genetics / Q000276:immunology; D014617:Vacuoles / Q000235:genetics / Q000276:immunology / Q000473:pathology
chemical_list: D000974:Antibodies, Antinuclear; D001324:Autoantigens; D015395:Histocompatibility Antigens Class I