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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MDPI AG
2016-09-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | http://www.mdpi.com/1422-0067/17/10/1625 |
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| author | Susanna Esposito Sophie Guez Annalisa Orenti Gianluca Tadini Giulietta Scuvera Laura Corti Alessia Scala Elia Biganzoli Emilio Berti Nicola Principi |
| author_facet | Susanna Esposito Sophie Guez Annalisa Orenti Gianluca Tadini Giulietta Scuvera Laura Corti Alessia Scala Elia Biganzoli Emilio Berti Nicola Principi |
| author_sort | Susanna Esposito |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-12T13:05:42Z |
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| issn | 1422-0067 |
| language | English |
| last_indexed | 2024-04-12T13:05:42Z |
| publishDate | 2016-09-01 |
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| spelling | doaj.art-348e800ce90d46e7b0842708ae3fd1682022-12-22T03:32:02ZengMDPI AGInternational Journal of Molecular Sciences1422-00672016-09-011710162510.3390/ijms17101625ijms17101625Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis BullosaSusanna Esposito0Sophie Guez1Annalisa Orenti2Gianluca Tadini3Giulietta Scuvera4Laura Corti5Alessia Scala6Elia Biganzoli7Emilio Berti8Nicola Principi9Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyPediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyPediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyPediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyPediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyDermatology Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyPediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyUnit of Medical Statistics, Biometry and Bioinformatics “G.A. Maccacaro”, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan 20122, ItalyDermatology Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyPediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan 20122, ItalyIn 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.http://www.mdpi.com/1422-0067/17/10/1625autoimmunityBirmingham Epidermolysis Bullosa Severity (BEBS) scoreepidermolysis bullosainflammatory responseinherited epidermolysis bullosa |
| spellingShingle | Susanna Esposito Sophie Guez Annalisa Orenti Gianluca Tadini Giulietta Scuvera Laura Corti Alessia Scala Elia Biganzoli Emilio Berti Nicola Principi Autoimmunity and Cytokine Imbalance in Inherited Epidermolysis Bullosa International Journal of Molecular Sciences autoimmunity Birmingham Epidermolysis Bullosa Severity (BEBS) score epidermolysis bullosa inflammatory response 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 | autoimmunity Birmingham Epidermolysis Bullosa Severity (BEBS) score epidermolysis bullosa inflammatory response inherited epidermolysis bullosa |
| url | http://www.mdpi.com/1422-0067/17/10/1625 |
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