title: Cutaneous Lupus Erythematosus: An Update on Pathogenesis, Diagnosis and Treatment.
abstract: Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic disease. Recent studies in this area continue to shape our understanding of this disease and treatment options. Epidemiologic studies have found an incidence of CLE of 4.30 per 100,000, which approaches similar analysis for systemic lupus erythematosus (SLE). Although there have been extensive efforts to define SLE, the classification of CLE and its subgroups remains a challenge. Currently, diagnosis relies on clinical and laboratory findings as well as skin histology. The Cutaneous Lupus Area and Severity Index™ (CLASI™) is a validated measure of disease activity and damage. CLE pathogenesis is multifactorial and includes genetic contributions as well as effects of ultraviolet (UV) light. Immune dysregulation and aberrant cell signaling pathways through cytokine cascades are also implicated. Patient education and avoidance of triggers are key to disease prevention. Antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for the treatment of recalcitrant disease.
authors.affiliation: Corporal Michael J. Crescenz Veterans Affairs Medical Center (Philadelphia), Philadelphia, PA, USA.|Department of Dermatology, Perelman Center for Advanced Medicine, Perelman School of Medicine, University of Pennsylvania, Suite 1-330A, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA.
authors.forename: Emily Z
authors.initials: EZ
authors.lastname: Hejazi
authors.affiliation: Corporal Michael J. Crescenz Veterans Affairs Medical Center (Philadelphia), Philadelphia, PA, USA. werth@mail.med.upenn.edu.|Department of Dermatology, Perelman Center for Advanced Medicine, Perelman School of Medicine, University of Pennsylvania, Suite 1-330A, 3400 Civic Center Boulevard, Philadelphia, PA, 19104, USA. werth@mail.med.upenn.edu.
authors.forename: Victoria P
authors.initials: VP
authors.lastname: Werth
mesh_terms: D000287:Administration, Topical; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000962:Antimalarials / Q000627:therapeutic use; D065095:Calcineurin Inhibitors / Q000627:therapeutic use; D016207:Cytokines / Q000235:genetics / Q000276:immunology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006680:HLA Antigens / Q000235:genetics; D006239:Haplotypes; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008178:Lupus Erythematosus, Cutaneous / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D012720:Severity of Illness Index; D013792:Thalidomide / Q000031:analogs & derivatives; D014466:Ultraviolet Rays / Q000009:adverse effects
chemical_list: D000911:Antibodies, Monoclonal; D000962:Antimalarials; D065095:Calcineurin Inhibitors; D016207:Cytokines; D005938:Glucocorticoids; D006680:HLA Antigens; D007166:Immunosuppressive Agents; D013792:Thalidomide