European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes

R Knobler; P Moinzadeh; N Hunzelmann; A Kreuter; A Cozzio; L Mouthon; M Cutolo; F Rongioletti; C P Denton; L Rudnicka; L A Frasin; V Smith; A Gabrielli; E Aberer; M Bagot; G Bali; J Bouaziz; A Braae Olesen; I Foeldvari; C Frances; A Jalili; U Just; V Kähäri; S Kárpáti; K Kofoed; D Krasowska; M Olszewska; C Orteu; J Panelius; A Parodi; A Petit; P Quaglino; A Ranki; J M Sanchez Schmidt; J Seneschal; A Skrok; M Sticherling; C Sunderkötter; A Taieb; A Tanew; P Wolf; M Worm; N J Wutte; T Krieg

doi:10.1111/jdv.14458

European Dermatology Forum S1-guideline 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. 2017 Sep;31(9):1401-1424. doi: 10.1111/jdv.14458. Epub 2017 Aug 9.

Authors

R Knobler¹, P Moinzadeh², N Hunzelmann², A Kreuter³, A Cozzio⁴, L Mouthon⁵, M Cutolo⁶, F Rongioletti⁷, C P Denton⁸, L Rudnicka⁹, L A Frasin¹⁰, V Smith¹¹, A Gabrielli¹², E Aberer¹³, M Bagot¹⁴, G Bali¹⁵, J Bouaziz¹⁴, A Braae Olesen¹⁶, I Foeldvari¹⁷, C Frances¹⁸, A Jalili¹⁹, U Just¹, V Kähäri²⁰, S Kárpáti¹⁵, K Kofoed²¹, D Krasowska²², M Olszewska⁹, C Orteu²³, J Panelius²⁴, A Parodi²⁵, A Petit¹⁴, P Quaglino²⁶, A Ranki²⁷, J M Sanchez Schmidt²⁸, J Seneschal²⁹, A Skrok⁹, M Sticherling³⁰, C Sunderkötter³¹, A Taieb²⁹, A Tanew¹, P Wolf¹³, M Worm³², N J Wutte¹³, T Krieg²

Affiliations

¹ Department of Dermatology, Medical University of Vienna, Vienna, Austria.
² Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany.
³ Department of Dermatology, Venereology and Allergology, HELIOS St. Elisabeth Klinik Oberhausen, University Witten-Herdecke, Oberhausen, Germany.
⁴ Department of Dermatology, Venereology and Allergology, Kantonsspital St. Gallen, St. Gallen, Switzerland.
⁵ Service de Médecine Interne, Centre de référence maladies rares: vascularites et sclérodermie systémique, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.
⁶ Research Laboratories and Academic Division of Clinical Rheumatology, IRCCS San Martino, University Medical School of Genoa, Genoa, Italy.
⁷ Dermatology Unit, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
⁸ Division of Medicine, Centre for Rheumatology, University College London, London, UK.
⁹ Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.
¹⁰ Dermatology Unit, Hospital of Lecco, Lecco, Italy.
¹¹ Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
¹² Department of Clinical and Molecular Science, Università Politecnica delle Marche, Ancona, Italy.
¹³ Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
¹⁴ Department of Dermatology, Hôpital Saint-Louis, Hôpitaux Universitaires, Paris, France.
¹⁵ Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
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 Department of Dermatology and Allergology, Hôpital Tenon, Assistance Publique Hôpitaux de Paris, Université Pierre et Marie Curie, Paris, France.
¹⁹ Division of Immunology, Allergy and Infectious Diseases, Department of Dermatology, Medical University of Vienna, Vienna, Austria.
²⁰ Department of Dermatology and Venereology, Turku University Hospital and University of Turku, Turku, Finland.
²¹ Department of Dermato-Allergology, Herlev and Gentofte University Hospital, Copenhagen, Denmark.
²² Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland.
²³ Department of Dermatology, Connective Tissue Diseases Service, Royal Free Hospital, London, UK.
²⁴ Department of Dermatology, Allergology and Venereology, University of Helsinki, and Skin and Allergy Hospital, Helsinki University Central Hospital, Helsinki, Finland.
²⁵ Department of Dermatology, IRCCS San Martino, University Medical School of Genoa, Genoa, Italy.
²⁶ Department of Medical Sciences, University of Turin, Turin, Italy.
²⁷ Department of Dermatology, Allergology and Venereology, Inflammation Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
²⁸ Department of Dermatology, Hospital del Mar-Parc de Salut Mar, Universitat Autònoma de Barcelona, Barcelona, Spain.
²⁹ Department of Dermatology and Pediatric Dermatology, National Centre for Rare Skin Disorders, Hôpital Saint-Andre, University of Bordeaux, Bordeaux, France.
³⁰ Department of Dermatology, University Hospital of Erlangen, Erlangen, Germany.
³¹ Department of Dermatology and Venereology, University Hospital Halle, Halle (Saale), Germany.
³² Department of Dermatology, Venereology and Allergology, University Hospital Charité - Universitätsmedizin Berlin, Berlin, Germany.

PMID: 28792092
DOI: 10.1111/jdv.14458

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 guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum.

Publication types

Practice Guideline

MeSH terms

Diagnosis, Differential
Europe
Humans
Physical Examination
Prognosis
Scleroderma, Localized* / diagnosis
Scleroderma, Localized* / pathology
Scleroderma, Localized* / therapy
Scleroderma, Systemic* / diagnosis
Scleroderma, Systemic* / pathology
Scleroderma, Systemic* / therapy
Undifferentiated Connective Tissue Diseases* / diagnosis
Undifferentiated Connective Tissue Diseases* / pathology
Undifferentiated Connective Tissue Diseases* / therapy