title: S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV).
abstract: Cutaneous lupus erythematosus (CLE) is a rare inflammatory autoimmune disease with heterogeneous clinical manifestations. To date, no therapeutic agents have been licensed specifically for patients with this disease entity, and topical and systemic drugs are mostly used 'off-label'. The aim of the present guideline was to achieve a broad consensus on treatment strategies for patients with CLE by a European subcommittee, guided by the European Dermatology Forum (EDF) and supported by the European Academy of Dermatology and Venereology (EADV). In total, 16 European participants were included in this project and agreed on all recommendations. Topical corticosteroids remain the mainstay of treatment for localized CLE, and further topical agents, such as calcineurin inhibitors, are listed as alternative first-line or second-line topical therapeutic option. Antimalarials are recommended as first-line and long-term systemic treatment in all CLE patients with severe and/or widespread skin lesions, particularly in patients with a high risk of scarring and/or the development of systemic disease. In addition to antimalarials, systemic corticosteroids are recommended as first-line treatment in highly active and/or severe CLE. Second- and third-line systemic treatments include methotrexate, retinoids, dapsone and mycophenolate mofetil or mycophenolate acid, respectively. Thalidomide should only be used in selected therapy-refractory CLE patients, preferably in addition to antimalarials. Several new therapeutic options, such as B-cell- or interferon α-targeted agents, need to be further evaluated in clinical trials to assess their efficacy and safety in the treatment of patients with CLE.
authors.affiliation: Interdisciplinary Center for Clinical Trials (IZKS), University Medical Center Mainz, Mainz, Germany.|Division of Immunogenetics, Tumor Immunology Program, German Cancer Research Center, Heidelberg, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Kuhn
authors.affiliation: Department of Dermatology, Medical University of Graz, Graz, Austria.
authors.forename: E
authors.initials: E
authors.lastname: Aberer
authors.affiliation: Department of Dermatology and Allergology, University of Szeged, Szeged, Hungary.
authors.forename: Z
authors.initials: Z
authors.lastname: Bata-Csörgő
authors.affiliation: Department of Medical and Surgical Critical Care Section of Dermatology, University of Florence, Florence, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Caproni
authors.affiliation: Evidence-Based Medicine Frankfurt, Institute for General Practice, Goethe-University Frankfurt, Frankfurt, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Dreher
authors.affiliation: Department of Dermatology and Allergology, Hôpital Tenon, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Frances
authors.affiliation: Department of Dermatology, Venerology and Allergology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Gläser
authors.affiliation: Department of Dermatology, Inselspital Bern - University Hospital, Bern, Switzerland.
authors.forename: H-W
authors.initials: HW
authors.lastname: Klötgen
authors.affiliation: Division of Immunogenetics, Tumor Immunology Program, German Cancer Research Center, Heidelberg, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Landmann
authors.affiliation: Department of Dermatology and Venereology, University Hospital Center Zagreb and School of Medicine University of Zagreb, Zagreb, Croatia.
authors.forename: B
authors.initials: B
authors.lastname: Marinovic
authors.affiliation: Institution for Clinical Sciences, Unit for Dermatology, Karolinska Institutet at Danderyd Hospital (KIDS), Stockholm, Sweden.
authors.forename: F
authors.initials: F
authors.lastname: Nyberg
authors.affiliation: Department of Dermatology, Colentina Clinical Hospital, Bucharest, Romania.
authors.forename: R
authors.initials: R
authors.lastname: Olteanu
authors.affiliation: Department of Skin and allergic diseases, Inflammation Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: A
authors.initials: A
authors.lastname: Ranki
authors.affiliation: Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Wroclaw, Poland.
authors.forename: J C
authors.initials: JC
authors.lastname: Szepietowski
authors.affiliation: Department of Dermatology, Donauspital, University affiliated Hospital, Vienna, Austria.
authors.forename: B
authors.initials: B
authors.lastname: Volc-Platzer
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000962:Antimalarials / Q000627:therapeutic use*; D001688:Biological Products / Q000627:therapeutic use; D065095:Calcineurin Inhibitors / Q000627:therapeutic use*; D032921:Consensus; D003622:Dapsone / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D000077269:Lenalidomide; D008178:Lupus Erythematosus, Cutaneous / Q000188:drug therapy*; D008727:Methotrexate / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000627:therapeutic use; D017410:Practice Guidelines as Topic; D012176:Retinoids / Q000008:administration & dosage / Q000627:therapeutic use*; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D000962:Antimalarials; D001688:Biological Products; D065095:Calcineurin Inhibitors; D007166:Immunosuppressive Agents; D012176:Retinoids; D013792:Thalidomide; D003622:Dapsone; D000077269:Lenalidomide; D009173:Mycophenolic Acid; D008727:Methotrexate