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

R Knobler; M Geroldinger-Simić; A Kreuter; N Hunzelmann; P Moinzadeh; F Rongioletti; C P Denton; L Mouthon; M Cutolo; V Smith; A Gabrielli; M Bagot; A B Olesen; I Foeldvari; A Jalili; V Kähäri; S Kárpáti; K Kofoed; M Olszewska; J Panelius; P Quaglino; J Seneschal; M Sticherling; C Sunderkötter; A Tanew; P Wolf; M Worm; A Skrok; L Rudnicka; T Krieg

doi:10.1111/jdv.19912

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

J Eur Acad Dermatol Venereol. 2024 Jul;38(7):1251-1280. doi: 10.1111/jdv.19912. Epub 2024 Mar 8.

Authors

R Knobler¹, M Geroldinger-Simić^{2

3}, A Kreuter⁴, N Hunzelmann⁵, P Moinzadeh⁵, F Rongioletti⁶, C P Denton⁷, L Mouthon^{8

9}, M Cutolo¹⁰, V Smith^{11

12

13}, A Gabrielli¹⁴, M Bagot¹⁵, A B Olesen¹⁶, I Foeldvari¹⁷, A Jalili¹⁸, V Kähäri¹⁹, S Kárpáti²⁰, K Kofoed²¹, M Olszewska²², J Panelius²³, P Quaglino²⁴, J Seneschal²⁵, M Sticherling²⁶, C Sunderkötter²⁷, A Tanew²⁸, P Wolf²⁹, M Worm³⁰, A Skrok²², L Rudnicka²², T Krieg³¹

Affiliations

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.

PMID: 38456584
DOI: 10.1111/jdv.19912

Abstract

The 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.

Publication types

Review

MeSH terms

Consensus*
Diagnosis, Differential
Humans
Scleroderma, Localized* / diagnosis
Scleroderma, Localized* / therapy
Scleroderma, Systemic* / diagnosis
Scleroderma, Systemic* / therapy

Grants and funding

Mallinckrodt Pharmaceuticals