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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-18881

Guiducci, S; Distler, J H W; Milia, A F; Miniati, I; Rogai, V; Manetti, M; Falcini, F; Ibba-Manneschi, L; Gay, S; Distler, O; Matucci-Cerinic, M (2009). Stiff skin syndrome: evidence for an inflammation-independent fibrosis? Rheumatology, 48(7):849-852.

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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-beta, connective tissue growth factor (CTGF), IL-6, -1beta, 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-1beta, -6 and MCP-1 was low, whereas VEGF was intensively expressed. No differences were observed for TGF-beta, 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.


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Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Center for Integrative Human Physiology
04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Date:1 July 2009
Deposited On:03 Jun 2009 10:49
Last Modified:05 Apr 2016 13:14
Publisher:Oxford University Press
Publisher DOI:10.1093/rheumatology/kep118
PubMed ID:19468049

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