EuroGuiderm guideline on lichen sclerosus—introduction into lichen sclerosus

Author:

Kirtschig G.1ORCID,Kinberger M.2ORCID,Kreuter A.3,Simpson R.4,Günthert A.5,van Hees C.6,Becker K.7,Ramakers M. J.8,Corazza M.9,Müller S.10,von Seitzberg S.11,Boffa M. J.12,Stein R.13,Barbagli G.14,Chi C. C.1516ORCID,Dauendorffer J. N.17ORCID,Fischer B.18,Gaskins M.2,Hiltunen‐Back E.19,Höfinger A.18,Köllmann N. H.18,Kühn H.20,Larsen H. K.21,Lazzeri M.22,Mendling W.23,Nikkels A. F.24,Promm M.25,Rall K. K.26,Regauer S.27,Sárdy M.28ORCID,Sepp N.29,Thune T.30,Tsiogka A.31ORCID,Vassileva S.32,Voswinkel L.20,Wölber L.33,Werner R. N.2ORCID

Affiliation:

1. Medbase Health Centre Frauenfeld Switzerland

2. Department of Dermatology, Venereology and Allergology, Division of Evidence‐ Based Medicine (dEBM) Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt‐Universität zu Berlin Berlin Germany

3. Department of Dermatology, Venereology, and Allergology HELIOS St. Elisabeth Hospital Oberhausen Oberhausen Germany

4. Centre of Evidence Based Dermatology University of Nottingham Nottingham UK

5. Gynäkologisches Tumorzentrum St. Anna Lucerne Switzerland

6. Department of Dermatology Erasmus University Medical Center Rotterdam The Netherlands

7. Office for Paediatric Surgery Bonn Germany

8. CenSeRe (Centre for Psychological, Relational, Sexual Health) Voorschoten The Netherlands

9. Section of Dermatology and Infectious Diseases, Department of Medical Sciences University of Ferrara Ferrara Italy

10. Department of Dermatology University Hospital Basel Basel Switzerland

11. The Danish Lichen Sclerosus Association Hørve Denmark

12. Department of Dermatology Mater Dei Hospital Msida Malta

13. Center for Pediatric, Adolescent and Reconstructive Urology, Medical Faculty Mannheim University of Medical Center Mannheim, Heidelberg University Mannheim Germany

14. Centro Chirurgico Toscano Arezzo Italy

15. Department of Dermatology Chang Gung Memorial Hospital Taoyuan Taiwan

16. College of Medicine, Chang Gung University Taoyuan Taiwan

17. Department of Dermatology, Centre for Genital and Sexually Transmitted Diseases University Hospital Saint Louis Paris France

18. The Swiss Lichen Sclerosus Association, Switzerland/Verein Lichen Sclerosus e.V. Dottikon Switzerland

19. Department of Dermatovenereology Helsinki University Hospital Helsinki Finland

20. The German Lichen Sclerosus Association Germany

21. Department of Dermatology and Venereology Copenhagen University Hospital, Bispebjerg Hospital Copenhagen Denmark

22. Department of Urology IRCCS Humanitas Research Hospital Rozzano (MI) Italy

23. German Center for Infections in Gynecology and Obstetrics at Helios University Hospital Wuppertal– University Witten/Herdecke Wuppertal Germany

24. Department of Dermatology University Medical Center of Liège Liège Belgium

25. Department of Paediatric Urology and Clinic St. Hedwig University Medical Centre of Regensburg Regensburg Germany

26. Department of Women's Health Women's University Hospital Tuebingen Tuebingen Germany

27. Diagnostic and Research Institute of Pathology Medical University Graz Graz Austria

28. Department of Dermatology, Venereology and Dermatooncology Semmelweis University Budapest Hungary

29. Department of Dermatology and Venereology Ordensklinikum Linz Elisabethinen Linz Austria

30. Department of Dermatology Haukeland University Hospital Bergen Norway

31. National and Kapodistrian University of Athens, Faculty of Medicine 1st Department of Dermatology‐Venereology, Andreas Sygros Hospital Athens Greece

32. Department of Dermatology and Venereology University Hospital “Alexandrovska”, Medical University – Sofia Sofia Bulgaria

33. Department of Gynaecology University Medical Centre Hamburg‐Eppendorf and Centre for Colposcopy and Vulvovaginal Disease Jersualem Hospital Hamburg Hamburg Germany

Abstract

AbstractIntroductionLichen 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 redness and oedema, typically followed by whitening of the genital skin; sometimes 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.ObjectivesThe guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), 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 MethodsThe 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.ResultsUltrapotent 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.ConclusionsLS 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.

Publisher

Wiley

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