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Review
. 2023 Mar 22:11:1129249.
doi: 10.3389/fped.2023.1129249. eCollection 2023.

Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement

Carmela Giancotta  1 , Nicole Colantoni  1 , Lucia Pacillo  1   2 , Veronica Santilli  1 , Donato Amodio  1 , Emma Concetta Manno  1 , Nicola Cotugno  1   2 , Gioacchino Andrea Rotulo  1   3 , Beatrice Rivalta  1   2 , Andrea Finocchi  1   2 , Caterina Cancrini  1   2 , Andrea Diociaiuti  4 , May El Hachem  4 , Paola Zangari  1
Affiliations
Review

Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement

Carmela Giancotta et al. Front Pediatr. .

Abstract

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.

Keywords: biologics; eczema; inborn errors of immunity; skin involvement; tailored treatment.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Warning signs for IEIs-A.
Figure 2
Figure 2
Proposed biologics in IEIs-A with skin involvement.
Figure 3
Figure 3
When to candidate for biologic treatment?
See this image and copyright information in PMC

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