Primary atopic disorders (PADs) are monogenic diseases characterized by allergy or atopy-related symptoms as fundamental features. In patients with PADs, primary immune deficiency and immune dysregulation symptoms are usually coexist. Chronic skin disease, manifesting with erythroderma, severe atopic dermatitis or eczema, and urticaria, is one of the main features observed in PADs, such as hyper-IgE syndromes, Omenn syndrome, Wiskott-Aldrich syndrome, IPEX-linked syndrome, skin barrier disorders, as well as some autoinflammatory diseases. The recognition of PADs in the context of an allergic phenotype is crucial to ensure prompt diagnosis and appropriate treatment. This article provides an overview of the main PADs with skin involvement.

Primary atopic disorders and chronic skin disease / Cinicola, BIANCA LAURA; Corrente, Stefania; Castagnoli, Riccardo; Lougaris, Vassilios; Giardino, Giuliana; Leonardi, Lucia; Volpi, Stefano; La Torre, Francesco; Federici, Silvia; Soresina, Annarosa; Cancrini, Caterina; Luigi Marseglia, Gian; Cardinale, Fabio. - In: PEDIATRIC ALLERGY AND IMMUNOLOGY. - ISSN 1399-3038. - (2022).

Primary atopic disorders and chronic skin disease

Bianca Laura Cinicola;Lucia Leonardi;
2022

Abstract

Primary atopic disorders (PADs) are monogenic diseases characterized by allergy or atopy-related symptoms as fundamental features. In patients with PADs, primary immune deficiency and immune dysregulation symptoms are usually coexist. Chronic skin disease, manifesting with erythroderma, severe atopic dermatitis or eczema, and urticaria, is one of the main features observed in PADs, such as hyper-IgE syndromes, Omenn syndrome, Wiskott-Aldrich syndrome, IPEX-linked syndrome, skin barrier disorders, as well as some autoinflammatory diseases. The recognition of PADs in the context of an allergic phenotype is crucial to ensure prompt diagnosis and appropriate treatment. This article provides an overview of the main PADs with skin involvement.
2022
IgE; allergy; inborn errors of immunity; primary atopic disorders; skin
01 Pubblicazione su rivista::01a Articolo in rivista
Primary atopic disorders and chronic skin disease / Cinicola, BIANCA LAURA; Corrente, Stefania; Castagnoli, Riccardo; Lougaris, Vassilios; Giardino, Giuliana; Leonardi, Lucia; Volpi, Stefano; La Torre, Francesco; Federici, Silvia; Soresina, Annarosa; Cancrini, Caterina; Luigi Marseglia, Gian; Cardinale, Fabio. - In: PEDIATRIC ALLERGY AND IMMUNOLOGY. - ISSN 1399-3038. - (2022).
File allegati a questo prodotto
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1639132
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 3
  • ???jsp.display-item.citation.isi??? 2
social impact