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Histopathological and molecular analysis in dermis and epidermis of patients with systemic and localized scleroderma
Pediatric Rheumatology volume 12, Article number: P304 (2014)
Introduction
Scleroderma is a highly complex disorder in its clinical manifestations and pathogenesis. It has a wide range of clinical manifestations due to varying degrees of vasculopathy, autoimmunity, altered endothelium function, and abnormal fibrosis which were accused in the pathogenesis of the disease.
Objectives
The aim of this study be made of the cases diagnosed in childhood with immune histochemical analysis is to shed light on the pathogenesis of the disease.
Methods
Single-blind clinical trial. The tissue samples obtained from patients PAS, hematoxylin and eosin, E-Cadherin, CTGF, Tunnel, and staining for TGF-β1 is applied and evaluated by light microscopy. Additionally, we have analyzed both TGFB1 level and mRNA expression analysis in plasma and tissue samples from patients. A total of 15 patients were enrolled in the study. These patients, who were chosen from, systemic (n = 8) or localized (n = 7) scleroderma patients clinically received, and for the disease of any systemic untreated and scleroderma other than skin disease.
Results
In the study a total of 22 tissue samples (15 diseased tissue, healthy tissue and 7) were used. The mean age of onset was 9.2 ± 1.2 years and the mean age of diagnosis was 15.3 ± 3.2 years. All patients with systemic sclerosis have antinuclear antibody (ANA) titer 1/160-1/640. ANA positivity in patients with localized scleroderma were no. Clinically with different characteristics in the two subgroups of the histopathology examination was shown to be distinct from the tissue level.
Conclusion
Playing a fundamental role in the pathogenesis of the TGF-B levels in both plasma and skin have been shown to be locally high. Particularly in patients with systemic scleroderma this elevation was found to be more pronounced. Treatment for this disease is still questionable results obtained will throw light on new treatment possibilities.
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None declared.
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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
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Sozeri, B., Aktug, H., Cetintas, V.B. et al. Histopathological and molecular analysis in dermis and epidermis of patients with systemic and localized scleroderma. Pediatr Rheumatol 12 (Suppl 1), P304 (2014). https://doi.org/10.1186/1546-0096-12-S1-P304
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DOI: https://doi.org/10.1186/1546-0096-12-S1-P304