title: Update on pathogenesis and treatment of CLE.
abstract: OBJECTIVE
  Cutaneous Lupus Erythematous (CLE) is an autoimmune disease in which patients may present with isolated skin findings or have CLE associated with underlying systemic disease. The most significant recent studies on its pathogenesis and therapeutic management are reviewed here.
  
  
  RESULTS
  Patients with subacute and Discoid Lupus Erythematous had elevated Interferon score, about a third of all cases of SCLE could be attributed to previous drug exposure, and smoking may be more closely associated with CLE than Systemic Lupus Erythematous (SLE). An underlying genetic defect in some subsets of CLE patients may also be shared with SLE. Efficacy of antimalarial therapy is enhanced by increasing treatment duration or maintaining higher blood drug concentrations. Combination antimalarials that include quinacrine, thalidomide analogs, and Mycophenalate Mofetil may also be effective in refractory CLE.
  
  
  CONCLUSIONS
  The pathogenesis of CLE remains unclear, and is likely multifactorial. Identified associations with subsets of CLE suggest future research questions in CLE pathogenesis. Subsets of CLE associated with interface dermatitis may share an underlying genetic defect in interferon signaling with SLE. The Cutaneous Lupus Disease Area and Severity Index is a valuable and widely used tool allowing standardized assessment and reporting of cutaneous disease activity and damage. More evidence is available to guide treatment of refractory CLE, but larger studies are needed.
  http://links.lww.com/COR/A4.
authors.affiliation: Philadelphia VA Medical Center, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.
authors.forename: Emily D
authors.initials: ED
authors.lastname: Privette
authors.forename: Victoria P
authors.initials: VP
authors.lastname: Werth
mesh_terms: D000279:Administration, Cutaneous; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000962:Antimalarials / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008178:Lupus Erythematosus, Cutaneous / Q000188:drug therapy* / Q000209:etiology* / Q000235:genetics; D012307:Risk Factors
chemical_list: D000911:Antibodies, Monoclonal; D000962:Antimalarials; D007166:Immunosuppressive Agents