[en] [en] INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness; it may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are usually a whitening of the genital skin, sometimes preceded by redness and oedema; fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early.
OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS (part 1), highlight important aspects in the care of LS patients, generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects.
MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives.
RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases.
CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.
Disciplines :
Dermatology
Author, co-author :
Kirtschig, G ; Medbase Health Centre, Frauenfeld, Switzerland
Kinberger, M ; Division of Evidence-Based Medicine (dEBM), Department of Dermatology, Venereology and Allergology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
Kreuter, A; Department of Dermatology, Venereology, and Allergology, HELIOS St. Elisabeth Hospital Oberhausen, Oberhausen, Germany
Simpson, R; Centre of Evidence Based Dermatology, University of Nottingham, Nottingham, UK
Günthert, A; Gynäkologisches Tumorzentrum St. Anna, Lucerne, Switzerland
van Hees, C; Department of Dermatology, Erasmus University Medical Center, Rotterdam, The Netherlands
Becker, K; Office for Paediatric Surgery, Bonn, Germany
Ramakers, M J; CenSeRe (Centre for Psychological, Relational, Sexual Health), Voorschoten, The Netherlands
Corazza, M; Section of Dermatology and Infectious Diseases, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
Müller, S ; Department of Dermatology, University Hospital Basel, Basel, Switzerland
von Seitzberg, S; The Danish Lichen Sclerosus Association, Hørve, Denmark
Boffa, M J; Department of Dermatology, Mater Dei Hospital, Msida, Malta
Stein, R; Center for Pediatric, Adolescent and Reconstructive Urology, Medical Faculty Mannheim, University of Medical Center Mannheim, Heidelberg University, Mannheim, Germany
Barbagli, G; Centro Chirurgico Toscano, Arezzo, Italy
Chi, C C ; Department of Dermatology, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan ; College of Medicine, Chang Gung University, Taoyuan, Taiwan
Dauendorffer, J N ; Department of Dermatology, Centre for Genital and Sexually Transmitted Diseases, University Hospital Saint Louis, Paris, France
Gaskins, M; Division of Evidence-Based Medicine (dEBM), Department of Dermatology, Venereology and Allergology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
Hiltunen-Back, E; Department of Dermatovenereology, Helsinki University Hospital, Helsinki, Finland
Köllmann, N H; The Swiss Lichen Sclerosus Association, Switzerland/Verein Lichen Sclerosus e.V., Dottikon, Switzerland
Kühn, H; The German Lichen Sclerosus Association, Hamburg, Germany
Larsen, H K ; Department of Dermatology and Venereology, Copenhagen University Hospital, Bispebjerg Hospital, Copenhagen, Denmark
Lazzeri, M; Department of Urology, IRCCS Humanitas Research Hospital, Rozzano, MI, Italy
Mendling, W; German Center for Infections in Gynecology and Obstetrics, Helios University Hospital Wuppertal-University Witten/Herdecke, Wuppertal, Germany
Nikkels, Arjen ; Université de Liège - ULiège > Département des sciences cliniques > Dermatologie
Promm, M; Department of Paediatric Urology and Clinic St. Hedwig, University Medical Centre of Regensburg, Regensburg, Germany
Rall, K K; Department of Women's Health, Women's University Hospital Tuebingen, Tuebingen, Germany
Regauer, S; Diagnostic and Research Institute of Pathology, Medical University Graz, Graz, Austria
Sárdy, M ; Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary
Sepp, N; Department of Dermatology and Venereology, Ordensklinikum Linz Elisabethinen, Linz, Austria
Thune, T; Department of Dermatology, Haukeland University Hospital, Bergen, Norway
Tsiogka, A ; 1st Department of Dermatology-Venereology, Faculty of Medicine, Andreas Sygros Hospital, National and Kapodistrian University of Athens, Athens, Greece
Vassileva, S; Department of Dermatology and Venereology, University Hospital "Alexandrovska", Medical University - Sofia, Sofia, Bulgaria
Voswinkel, L; The German Lichen Sclerosus Association, Hamburg, Germany
Wölber, L; Department of Gynaecology, University Medical Centre Hamburg-Eppendorf and Centre for Colposcopy and Vulvovaginal Disease Jersualem Hospital Hamburg, Hamburg, Germany
Werner, R N ; Division of Evidence-Based Medicine (dEBM), Department of Dermatology, Venereology and Allergology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
This EuroGuiDerm guideline was funded through the EuroGuiDerm Centre for Guideline Development. The European Dermatology Forum is responsible for fundraising and holds all raised funds in one account. The EuroGuiDerm Team is not involved in fundraising or in the decision making on which guideline (GL) or consensus statement (CS) development is funded. The decisions on which GL/CS is funded are made by the EuroGuiDerm Board of Directors independently. The EDF or any other body supporting the EuroGuiDerm is never involved in the guideline development and had no say on the content or focus of the guideline.
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