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Open Access Research article

Histopathological cutaneous alterations in systemic sclerosis: a clinicopathological study

Jens T Van Praet1*, Vanessa Smith1, Marc Haspeslagh23, Nele Degryse1, Dirk Elewaut1 and Filip De Keyser1

Author Affiliations

1 Department of Rheumatology, Ghent University Hospital, De Pintelaan 185, BE-9000 Gent, Belgium

2 Department of Dermatology, Ghent University Hospital, De Pintelaan 185, BE-9000 Gent, Belgium

3 Department of Pathology, Stedelijk Ziekenhuis Roeselare, Brugsesteenweg 90, BE-8800 Roeselare, Belgium

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Arthritis Research & Therapy 2011, 13:R35  doi:10.1186/ar3267

Published: 28 February 2011

Abstract

Introduction

The aims of the present study were to identify histopathological parameters which are linked to local clinical skin disease at two distinct anatomical sites in systemic sclerosis (SSc) patients with skin involvement (limited cutaneous systemic sclerosis (lcSSc) or diffuse cutaneous systemic sclerosis (dcSSc)) and to determine the sensitivity of SSc specific histological alterations, focusing on SSc patients without clinical skin involvement (limited SSc (lSSc)).

Methods

Histopathological alterations were systematically scored in skin biopsies of 53 consecutive SSc patients (dorsal forearm and upper inner arm) and 18 controls (upper inner arm). Clinical skin involvement was evaluated using the modified Rodnan skin score. In patients with lcSSc or dcSSc, associations of histopathological parameters with local clinical skin involvement were determined by generalised estimation equation modelling.

Results

The hyalinised collagen score, the myofibroblast score, the mean epidermal thickness, the mononuclear cellular infiltration and the frequency of focal exocytosis differed significantly between biopsies with and without local clinical skin involvement. Except for mononuclear cellular infiltration, all of the continuous parameters correlated with the local clinical skin score at the dorsal forearm. Parakeratosis, myofibroblasts and intima proliferation were present in a minority of the SSc biopsies, but not in controls. No differences were found between lSSc and controls.

Conclusions

Several histopathological parameters are linked to local clinical skin disease. SSc-specific histological alterations have a low diagnostic sensitivity.