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Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement
Inborn errors of immunity associated with atopy (IEIs-A) are a group of inherited monogenic disorders that occur with immune dysregulation and frequent skin involvement. Several pathways are involved in the pathogenesis of these conditions, including immune system defects, alterations of skin barrie...
Autores principales: | , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10073443/ https://www.ncbi.nlm.nih.gov/pubmed/37033173 http://dx.doi.org/10.3389/fped.2023.1129249 |
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author | Giancotta, Carmela Colantoni, Nicole Pacillo, Lucia Santilli, Veronica Amodio, Donato Manno, Emma Concetta Cotugno, Nicola Rotulo, Gioacchino Andrea Rivalta, Beatrice Finocchi, Andrea Cancrini, Caterina Diociaiuti, Andrea El Hachem, May Zangari, Paola |
author_facet | Giancotta, Carmela Colantoni, Nicole Pacillo, Lucia Santilli, Veronica Amodio, Donato Manno, Emma Concetta Cotugno, Nicola Rotulo, Gioacchino Andrea Rivalta, Beatrice Finocchi, Andrea Cancrini, Caterina Diociaiuti, Andrea El Hachem, May Zangari, Paola |
author_sort | Giancotta, Carmela |
collection | PubMed |
description | Inborn errors of immunity associated with atopy (IEIs-A) are a group of inherited monogenic disorders that occur with immune dysregulation and frequent skin involvement. Several pathways are involved in the pathogenesis of these conditions, including immune system defects, alterations of skin barrier and metabolism perturbations. Current technological improvements and the higher accessibility to genetic testing, recently allowed the identification of novel molecular pathways involved in IEIs-A, also informing on potential tailored therapeutic strategies. Compared to other systemic therapy for skin diseases, biologics have the less toxic and the best tolerated profile in the setting of immune dysregulation. Here, we review IEIs-A with skin involvement focusing on the tailored therapeutic approach according to their pathogenetic mechanism. |
format | Online Article Text |
id | pubmed-10073443 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-100734432023-04-06 Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement Giancotta, Carmela Colantoni, Nicole Pacillo, Lucia Santilli, Veronica Amodio, Donato Manno, Emma Concetta Cotugno, Nicola Rotulo, Gioacchino Andrea Rivalta, Beatrice Finocchi, Andrea Cancrini, Caterina Diociaiuti, Andrea El Hachem, May Zangari, Paola Front Pediatr Pediatrics Inborn errors of immunity associated with atopy (IEIs-A) are a group of inherited monogenic disorders that occur with immune dysregulation and frequent skin involvement. Several pathways are involved in the pathogenesis of these conditions, including immune system defects, alterations of skin barrier and metabolism perturbations. Current technological improvements and the higher accessibility to genetic testing, recently allowed the identification of novel molecular pathways involved in IEIs-A, also informing on potential tailored therapeutic strategies. Compared to other systemic therapy for skin diseases, biologics have the less toxic and the best tolerated profile in the setting of immune dysregulation. Here, we review IEIs-A with skin involvement focusing on the tailored therapeutic approach according to their pathogenetic mechanism. Frontiers Media S.A. 2023-03-22 /pmc/articles/PMC10073443/ /pubmed/37033173 http://dx.doi.org/10.3389/fped.2023.1129249 Text en © 2023 Giancotta, Colantoni, Pacillo, Santilli, Amodio, Manno, Cotugno, Rotulo, Rivalta, Finocchi, Cancrini, Diociaiuti, El Hachem and Zangari. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Giancotta, Carmela Colantoni, Nicole Pacillo, Lucia Santilli, Veronica Amodio, Donato Manno, Emma Concetta Cotugno, Nicola Rotulo, Gioacchino Andrea Rivalta, Beatrice Finocchi, Andrea Cancrini, Caterina Diociaiuti, Andrea El Hachem, May Zangari, Paola Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement |
title | Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement |
title_full | Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement |
title_fullStr | Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement |
title_full_unstemmed | Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement |
title_short | Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement |
title_sort | tailored treatments in inborn errors of immunity associated with atopy (ieis-a) with skin involvement |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10073443/ https://www.ncbi.nlm.nih.gov/pubmed/37033173 http://dx.doi.org/10.3389/fped.2023.1129249 |
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