Primary atopic disorders and chronic skin disease. (25th January 2022)

• Record Type: Journal Article
• Authors: Cinicola, Bianca Laura; Corrente, Stefania; Castagnoli, Riccardo; Lougaris, Vassilios; Giardino, Giuliana; Leonardi, Lucia; Volpi, Stefano; La Torre, Francesco; Federici, Silvia; Soresina, Annarosa; Cancrini, Caterina; Marseglia, Gian Luigi; Cardinale, Fabio
• Editors: Licari, Amelia
• Is Part Of: Pediatric allergy and immunology. Volume 33(2022)Supplement 27
• Page Start: 65
• Page End: 68
• Publication Date: 2022-01-25
• Subjects: allergy -- IgE -- inborn errors of immunity -- primary atopic disorders -- skin; Allergy in children -- Periodicals; Immunologic diseases in children -- Periodicals; 617
• DOI: 10.1111/pai.13633
• Languages: English
• ISSNs: 0905-6157

Abstract

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.