Update on pathogenesis and treatment of CLEPrivette, Emily D.a , b; Werth, Victoria P.a , bCurrent Opinion in Rheumatology: September 2013 - Volume 25 - Issue 5 - p 584–590 doi: 10.1097/BOR.0b013e32836437ba SYSTEMIC LUPUS ERYTHEMATOSUS AND SJOGREN SYNDROME: Edited by Mariana Kaplan Abstract Author Information Purpose of review 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. Recent findings 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. Summary 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. Video abstract http://links.lww.com/COR/A4. aPhiladelphia VA Medical Center bDepartment of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA Correspondence to Dr Victoria P. Werth, MD, Department of Dermatology, Perelman Center for Advanced Medicine, Suite 1-330A, 3400 Civic Center Boulevard, Philadelphia, PA 19104, USA. Tel: +1 215 823 4208; fax: +1 866 755 0625; e-mail: email@example.com Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.co-rheumatology.com). © 2013 Lippincott Williams & Wilkins, Inc.