Fulltext

Stiff skin syndrome: evidence for an inflammation-independent fibrosis?

Guiducci, Serena ; Distler, Joerg H. W. ; Milia, Anna Franca ; Miniati, Irene ; Rogai, Veronica ; Manetti, Mirko ; Falcini, Fernanda ; Ibba-Manneschi, Lidia ; Gay, Steffen ; Distler, Oliver ; Matucci-Cerinic, Marco

In: Rheumatology, 2009, vol. 48, no. 7, p. 849-852

Ajouter à la liste personnelle
    Titre
    • Stiff skin syndrome: evidence for an inflammation-independent fibrosis?
    Auteur
    • Guiducci, Serena. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Distler, Joerg H. W.. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Milia, Anna Franca. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Miniati, Irene. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Rogai, Veronica. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Manetti, Mirko. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Falcini, Fernanda. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Ibba-Manneschi, Lidia. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Gay, Steffen. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Distler, Oliver. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    • Matucci-Cerinic, Marco. Department of BioMedicine, Division of Rheumatology and DENOthe Centre, University of Florence, AOUC, Florence, Italy,Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland,Department of Anatomy, Histology and Forensic Medicine and Department of Pediatrics, Rheumatology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:297845
    Summary
    Objectives. Stiff skin syndrome (SSS) is a rare scleroderma-like syndrome of unknown aetiology. A 16-year-old boy presented with thoracic and abdominal asymmetry, and ‘orange peel' cutaneous lesions, with fibrotic stone-hard indurations at the buttocks, thighs and arms leading to secondary joint contractures of the extremities. Our aim was to analyse the expression of extracellular matrix (ECM) molecules and pro-fibrotic cytokines in the dermis and epidermis of SSS. Methods. The diagnosis of SSS was confirmed by clinical and histopathological examination. Collagen type 1 alpha-2 chain (Col1A2), fibronectin-1, thrombospondin-1, TGF-β, connective tissue growth factor (CTGF), IL-6, -1β, ET-1, Fibroblast growth factor receptor 3 (FGFR-3) and MCP-1 expression was analysed in SSS and age- and sex-matched healthy control skin by real-time PCR. VEGF expression was also studied. Results. Histopathological examination showed flattened dermal papillae, a scarce presence of sub-epidermal microvessels and mild dermal fibrosis, but no inflammatory infiltrates. In the SSS dermis, the expression of IL-1β, -6 and MCP-1 was low, whereas VEGF was intensively expressed. No differences were observed for TGF-β, CTGF and ET-1. In contrast, col1A2, fibronectin-1 and thrombospondin-1 were overexpressed in the SSS dermis. Conclusion. In our SSS patient, an overexpression of ECM proteins was detected, whereas no inflammatory infiltrates or up-regulation of pro-fibrotic cytokines were found. The data suggest that fibrosis in SSS might be independent from inflammation