Primary Atopic Disorders

Primary atopic disorders describes a series of monogenic diseases that have allergy- or atopic effector-related symptoms as a substantial feature. The underlying pathogenic genetic lesions help illustrate fundamental pathways in atopy, opening up diagnostic and therapeutic options for further study...

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Bibliographic Details
Published inAnnual review of immunology Vol. 38; no. 1; pp. 785 - 808
Main Author Milner, Joshua D
Format Journal Article
LanguageEnglish
Published United States Annual Reviews 26.04.2020
Subjects
allergy
genetics
mast cell function
monogenic
primary atopic disorders
skin barrier
Th2
skin barrier
primary atopic disorders
genetics
mast cell function
allergy
Th2
monogenic
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Summary:Primary atopic disorders describes a series of monogenic diseases that have allergy- or atopic effector-related symptoms as a substantial feature. The underlying pathogenic genetic lesions help illustrate fundamental pathways in atopy, opening up diagnostic and therapeutic options for further study in those patients, but ultimately for common allergic diseases as well. Key pathways affected in these disorders include T cell receptor and B cell receptor signaling, cytokine signaling, skin barrier function, and mast cell function, as well as pathways that have not yet been elucidated. While comorbidities such as classically syndromic presentation or immune deficiency are often present, in some cases allergy alone is the presenting symptom, suggesting that commonly encountered allergic diseases exist on a spectrum of monogenic and complex genetic etiologies that are impacted by environmental risk factors.
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ISSN:0732-0582
1545-3278
DOI:10.1146/annurev-immunol-042718-041553