Primary atopic disorders and chronic skin disease
Bianca Laura Cinicola, S Corrente, Riccardo Castagnoli, Vassilios Lougaris, Giuliana Giardino, Lucia Leonardi, Stefano Volpi, Francesco La Torre, Silvia Federici, Annarosa Soresina, Caterina Cancrini, Gian Luigi Marseglia, Fabio Cardinale, the Immunology Task Force of the Italian Society of Pediatric Allergy, Immunology (SIAIP)
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.