Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes

Author:

Knobler R.1ORCID,Geroldinger‐Simić M.23,Kreuter A.4,Hunzelmann N.5,Moinzadeh P.5ORCID,Rongioletti F.6ORCID,Denton C. P.7ORCID,Mouthon L.89,Cutolo M.10,Smith V.111213,Gabrielli A.14,Bagot M.15ORCID,Olesen A. B.16,Foeldvari I.17,Jalili A.18ORCID,Kähäri V.19,Kárpáti S.20,Kofoed K.21,Olszewska M.22,Panelius J.23,Quaglino P.24ORCID,Seneschal J.25ORCID,Sticherling M.26,Sunderkötter C.27,Tanew A.28ORCID,Wolf P.29,Worm M.30,Skrok A.22,Rudnicka L.22ORCID,Krieg T.31

Affiliation:

1. Department of Dermatology Medical University of Vienna Vienna Austria

2. Department of Dermatology Ordensklinikum Linz Elisabethinen Linz Austria

3. Faculty of Medicine Johannes Kepler University Linz Austria

4. Department of Dermatology, Venereology and Allergology, HELIOS St. Elisabeth Klinik Oberhausen University Witten‐Herdecke Oberhausen Germany

5. Department of Dermatology and Venereology University of Cologne Cologne Germany

6. Vita Salute University IRCSS San Raffaele Hospital Milan Italy

7. Center for Rheumatology Royal Free and University College Medical School London UK

8. Service de Médecine Interne, Centre de Référence Maladies Auto‐Immunes et Systémiques Rares d'Ile de France APHP‐CUP, Hôpital Cochin Paris France

9. Institut Cochin Université de Paris Cité Paris France

10. Laboratories for Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI University Medical School of Genoa, IRCCS San Martino Genoa Genoa Italy

11. Department of Internal Medicine Ghent University Ghent Belgium

12. Department of Rheumatology Ghent University Hospital Ghent Belgium

13. Unit for Molecular Immunology and Inflammation VIB Inflammation Research Center (IRC) Ghent Belgium

14. Fondazione di Medicina Molecolare e Terapia Cellulare Università Politecnica delle Marche Ancona Italy

15. Department of Dermatology, Hôpital Saint‐Louis Université Paris Cité Paris France

16. Department of Dermatology University Hospital of Aarhus Aarhus Denmark

17. Hamburg Centre for Pediatric and Adolescent Rheumatology Schön Klinik Hamburg Eilbek Hamburg Germany

18. Dermatology & Skin Care Clinic Buochs Switzerland

19. Department of Dermatology and Venereology University of Turku and Turku University Hospital Turku Finland

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

21. The Skin Clinic Copenhagen Denmark

22. Department of Dermatology Medical University of Warsaw Warsaw Poland

23. Department of Dermatology and Allergology University of Helsinki and Helsinki University Hospital Helsinki Finland

24. Department of Medical Sciences, Dermatologic Clinic University of Turin Turin Italy

25. Department of Dermatology and Pediatric Dermatology, National Centre for Rare Skin Disorders, Hôpital Saint‐Andre University of Bordeaux, CNRS, Immuno CencEpT, UMR 5164 Bordeaux France

26. Department of Dermatology Universitätsklinikum Erlangen Erlangen Germany

27. Department of Dermatology and Venereology University Hospital Halle Halle (Saale) Germany

28. Private Practice Vienna Austria

29. Department of Dermatology Medical University of Graz Graz Austria

30. Division of Allergy and Immunology, Department of Dermatology, Venereology and Allergology University Hospital Charité – Universitätsmedizin Berlin Berlin Germany

31. Department of Dermatology and Venereology, and Translational Matrix Biology University of Cologne Cologne Germany

Abstract

AbstractThe term ‘sclerosing diseases of the skin’ comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first‐ and advanced‐line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes.

Funder

Mallinckrodt Pharmaceuticals

Publisher

Wiley

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