Localized Scleroderma Is Associated with an Increased Risk of Systemic Sclerosis: A Matched Cohort Study

Authors

  • Bastian Aschoff Lübeck Institute of Experimental Dermatology, University of Lübeck and University Clinic Schleswig Holstein (UKSH), Lübeck, Germany https://orcid.org/0009-0002-4691-6762
  • Gabriela Riemekasten Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany https://orcid.org/0000-0002-5406-2464
  • Sören Dräger Department of Dermatology, University Clinic Schleswig Holstein (UKSH), Lübeck, Germany https://orcid.org/0000-0002-3701-8365
  • Ralf J. Ludwig Lübeck Institute of Experimental Dermatology, University of Lübeck and University Clinic Schleswig Holstein (UKSH), Lübeck, Germany; Institute and Comprehensive Center for Inflammation Medicine, University of Lübeck, Lübeck, Germany https://orcid.org/0000-0002-1394-1737
  • Philip Curman Lübeck Institute of Experimental Dermatology, University of Lübeck and University Clinic Schleswig Holstein (UKSH), Lübeck, Germany; Dermato-Venereology Clinic, Karolinska University Hospital, Stockholm, Sweden; Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; Dermatology and Venereology Division, Department of Medicine (Solna), Karolinska Institutet, Stockholm, Sweden https://orcid.org/0000-0003-2051-8491

DOI:

https://doi.org/10.2340/actadv.v106.adv-2026-0759

Keywords:

Localized scleroderma, Systemic sclerosis, Epidemiology, Matched cohort study

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References

Papara C, De Luca DA, Bieber K, Vorobyev A, Ludwig RJ. Morphea: The 2023 update. Front Med (Lausanne) 2023; 10: 1108623. DOI: https://doi.org/10.3389/fmed.2023.1108623

Lipsker D, Bessis D, Cosnes A, Kluger N, Lutz V, Sauleau E, et al. Prospective evaluation of frequency of signs of systemic sclerosis in 76 patients with morphea. Clin Exp Rheumatol 2015; 33: S23–5.

Giuggioli D, Colaci M, Cocchiara E, Spinella A, Lumetti F, Ferri C. From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution. Dermatol Res Pract 2018; 2018: 1284687. DOI: https://doi.org/10.1155/2018/6984282

Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, et al. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis. J Eur Acad Dermatol Venereol 2017; 31: 1581–1594. DOI: https://doi.org/10.1111/jdv.14466

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Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 2011; 64: 217–228. DOI: https://doi.org/10.1016/j.jaad.2010.05.045

De Rosa C, Di Marco G, Bottino V, Pesce N, Brunetti AP, Stabile G, et al. Are systemic sclerosis and localized scleroderma (morphea) part of a common disease spectrum? A systematic review on their coexistence. Clin Dermatol 2025; 43: 850–858. DOI: https://doi.org/10.1016/j.clindermatol.2025.09.032

Ludwig RJ, Anson M, Zirpel H, Thaci D, Olbrich H, Bieber K, et al. A comprehensive review of methodologies and application to use the real-world data and analytics platform TriNetX. Front Pharmacol 2025; 16: 1516126. DOI: https://doi.org/10.3389/fphar.2025.1516126

Lipsitch M, Tchetgen Tchetgen E, Cohen T. Negative controls: a tool for detecting confounding and bias in observational studies. Epidemiology 2010; 21: 383–388. DOI: https://doi.org/10.1097/EDE.0b013e3181d61eeb

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Published

2026-10-06

How to Cite

Aschoff, B., Riemekasten, G., Dräger, S., Ludwig, R. J., & Curman, P. (2026). Localized Scleroderma Is Associated with an Increased Risk of Systemic Sclerosis: A Matched Cohort Study. Acta Dermato-Venereologica, 106, adv–2026. https://doi.org/10.2340/actadv.v106.adv-2026-0759

Issue

Vol. 106 (2026)

Section

Short Communication

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Copyright (c) 2026 Bastian Aschoff, Gabriela Riemekasten, Sören Dräger, Ralf J. Ludwig, Philip Curman

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This work is licensed under a Creative Commons Attribution 4.0 International License.

All digitalized ActaDV contents is available freely online. The Society for Publication of Acta Dermato-Venereologica owns the copyright for all material published until volume 88 (2008) and as from volume 89 (2009) the journal has been published fully Open Access, meaning the authors retain copyright to their work.

Unless otherwise specified, all Open Access articles are published under CC-BY-NC licences, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material for non-commercial purposes, provided proper attribution to the original work.

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