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 a-targeted agents, need to be further evaluated in clinical trials to assess their efficacy and safety in the treatment of patients with CLE. JEADVAll authors are participants of the European Society for Cutaneous Lupus Erythematosus (EUSCLE), which received a grant by the European Academy of Dermatology and Venereology (EADV) to perform the project. This grant was used to organize the consensus conferences and to reimburse the travel fees and the accommodation of each participant. In addition, the grant by the EADV was used to partly reimburse the personnel costs of Aysche Landmann for coordination of the project; and drafting, copy-editing and formatting of the manuscript. Elisabeth Aberer, Zsuszanna Bata-Cs€ org€ o, Marcia Caproni, Andreas Dreher, Camille Frances, Regine Gl€ aser, Hans-Wilhelm Kl€ otgen, Annegret Kuhn, Aysche Landmann, Branka Marinovic, Filippa Nyberg, Rodica Olteanu, Annamari Ranki and Beatrix Volc-Platzer have no conflicts of interest with regard to fees for participation in review activities, such as data monitoring boards, statistical analysis, or end point committees. Jacek C. Szepietowski participated in the Novartis Steering Committee and the Sandoz Data Monitoring Committee. Elisabeth Aberer, Zsuszanna Bata-Cs€ org€ o, Marcia Caproni, Andreas Dreher, Camille Frances, Regine Gl€ aser, Hans-Wilhelm Kl€ otgen, Annegret Kuhn, Branka Marinovic, Filippa Nyberg, Rodica Olteanu, Annamari Ranki, Jacek C. Szepietowski and Beatrix Volc-Platzer have no confl...

Practice‐based differences in paediatric discoid lupus erythematosus

“…Rheumatologists were more likely than dermatologists to ‘always initiate’ this first‐line for children with DLE. European consensus guidelines for adults with DLE have recommended hydroxychloroquine as first‐line for generalized disease, or for those at risk for systemic disease or significant scarring . Hydroxychloroquine has also been shown to reduce autoantibody accumulation and end‐organ damage in patients later diagnosed with SLE .…”

“…In addition, although European consensus guidelines were recently published for adults with cutaneous lupus, this work does not include guidelines for the management of paediatric patients. Optimal treatment strategies in children have not been systematically investigated.…”

Pregnancy and lupus erythematosus – a therapeutic challenge

“…Hydroxychloroquine is considered safe and thus recommended as fi rst-line treatment [ 8 ] . Hydroxychloroquine is considered safe and thus recommended as fi rst-line treatment [ 8 ] .…”

“…Hydroxychloroquine is considered safe and thus recommended as fi rst-line treatment [ 8 ] . If corticosteroids are required, a low daily dose should be chosen; the recommended maintenance dose for prednisolone is 10-15 mg/d [ 8 ] . If corticosteroids are required, a low daily dose should be chosen; the recommended maintenance dose for prednisolone is 10-15 mg/d [ 8 ] .…”

“…Methotrexate was discontinued fi ve months before the fi rst pregnancy, due its known teratogenicity and the patient's desire to have children [ 8 ] . Methotrexate was discontinued fi ve months before the fi rst pregnancy, due its known teratogenicity and the patient's desire to have children [ 8 ] .…”