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Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa

In order to evaluate the serum anti-skin autoantibodies and cytokine concentrations in patients with different epidermolysis bullosa (EB) types and severity, 42 EB patients and 38 controls were enrolled. Serum anti-skin antibodies were significantly higher in the patients than in the controls (p = 0...

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Autores principales: Esposito, Susanna, Guez, Sophie, Orenti, Annalisa, Tadini, Gianluca, Scuvera, Giulietta, Corti, Laura, Scala, Alessia, Biganzoli, Elia, Berti, Emilio, Principi, Nicola
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2016
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5085658/
https://www.ncbi.nlm.nih.gov/pubmed/27669234
http://dx.doi.org/10.3390/ijms17101625
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author Esposito, Susanna
Guez, Sophie
Orenti, Annalisa
Tadini, Gianluca
Scuvera, Giulietta
Corti, Laura
Scala, Alessia
Biganzoli, Elia
Berti, Emilio
Principi, Nicola
author_facet Esposito, Susanna
Guez, Sophie
Orenti, Annalisa
Tadini, Gianluca
Scuvera, Giulietta
Corti, Laura
Scala, Alessia
Biganzoli, Elia
Berti, Emilio
Principi, Nicola
author_sort Esposito, Susanna
collection PubMed
description In order to evaluate the serum anti-skin autoantibodies and cytokine concentrations in patients with different epidermolysis bullosa (EB) types and severity, 42 EB patients and 38 controls were enrolled. Serum anti-skin antibodies were significantly higher in the patients than in the controls (p = 0.008, p < 0.001, p < 0.001, p < 0.001 and p < 0.001 for desmoglein 1 (DSG1) desmoglein 3 (DSG3), bullous pemphigoid 180 (BP180), BP230 and type VII collagen (COL7), respectively). The same trend was observed for interleukin (IL)-1β, IL-2, IL-6, IL-10, tumor necrosis factor-β, and interferon-γ (p < 0.001, p < 0.001, p < 0.001, p = 0.008, p < 0.001 and p = 0.002, respectively). Increases in anti-skin antibodies and cytokine concentrations were higher in patients with recessive dystrophic EB than in those with different types of EB, in generalized cases than in localized ones, and in patients with higher Birmingham Epidermolysis Bullosa Severity (BEBS) scores than in those with a lower score. The BEBS score was directly correlated with BP180, BP230, COL7 (p = 0.015, p = 0.008 and p < 0.001, respectively) and IL-6 (p = 0.03), whereas IL-6 appeared significantly associated with DSG1, DSG3, BP180, BP230 and COL7 (p = 0.015, p = 0.023, p = 0.023, p = 0.015 and p = 0.005, respectively). This study showed that autoimmunity and inflammatory responses are frequently activated in EB, mainly in severe forms, suggesting the use of immunosuppressive drugs or biologicals that are active against pro-inflammatory cytokines to reduce clinical signs and symptoms of disease.
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spelling pubmed-50856582016-11-01 Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa Esposito, Susanna Guez, Sophie Orenti, Annalisa Tadini, Gianluca Scuvera, Giulietta Corti, Laura Scala, Alessia Biganzoli, Elia Berti, Emilio Principi, Nicola Int J Mol Sci Article In order to evaluate the serum anti-skin autoantibodies and cytokine concentrations in patients with different epidermolysis bullosa (EB) types and severity, 42 EB patients and 38 controls were enrolled. Serum anti-skin antibodies were significantly higher in the patients than in the controls (p = 0.008, p < 0.001, p < 0.001, p < 0.001 and p < 0.001 for desmoglein 1 (DSG1) desmoglein 3 (DSG3), bullous pemphigoid 180 (BP180), BP230 and type VII collagen (COL7), respectively). The same trend was observed for interleukin (IL)-1β, IL-2, IL-6, IL-10, tumor necrosis factor-β, and interferon-γ (p < 0.001, p < 0.001, p < 0.001, p = 0.008, p < 0.001 and p = 0.002, respectively). Increases in anti-skin antibodies and cytokine concentrations were higher in patients with recessive dystrophic EB than in those with different types of EB, in generalized cases than in localized ones, and in patients with higher Birmingham Epidermolysis Bullosa Severity (BEBS) scores than in those with a lower score. The BEBS score was directly correlated with BP180, BP230, COL7 (p = 0.015, p = 0.008 and p < 0.001, respectively) and IL-6 (p = 0.03), whereas IL-6 appeared significantly associated with DSG1, DSG3, BP180, BP230 and COL7 (p = 0.015, p = 0.023, p = 0.023, p = 0.015 and p = 0.005, respectively). This study showed that autoimmunity and inflammatory responses are frequently activated in EB, mainly in severe forms, suggesting the use of immunosuppressive drugs or biologicals that are active against pro-inflammatory cytokines to reduce clinical signs and symptoms of disease. MDPI 2016-09-24 /pmc/articles/PMC5085658/ /pubmed/27669234 http://dx.doi.org/10.3390/ijms17101625 Text en © 2016 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC-BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Esposito, Susanna
Guez, Sophie
Orenti, Annalisa
Tadini, Gianluca
Scuvera, Giulietta
Corti, Laura
Scala, Alessia
Biganzoli, Elia
Berti, Emilio
Principi, Nicola
Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa
title Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa
title_full Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa
title_fullStr Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa
title_full_unstemmed Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa
title_short Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa
title_sort autoimmunity and cytokine imbalance in inherited epidermolysis bullosa
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5085658/
https://www.ncbi.nlm.nih.gov/pubmed/27669234
http://dx.doi.org/10.3390/ijms17101625
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