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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
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.
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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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