Inborn errors of immunity (IEIs) are traditionally best known for enhanced susceptibility to infections. However, allergic inflammation, among other types of immune dysregulation, occurs frequently in IEIs. As such, the term primary atopic disorders (PADs) was recently coined to describe the group of heritable monogenic allergic disorders. It is becoming increasingly important for clinicians to recognize that allergic diseases such as food allergy, atopic dermatitis, and allergic asthma are expressions of misdirected immunity, and in patients who present with severe, early onset or coexisting allergic conditions, these can be indications of an underlying PAD. Identifying monogenic allergic disease through next-generation sequencing (NGS) can dramatically improve outcomes by allowing the use of precision-based therapy that targets the patient’s underlying molecular defect. It is therefore imperative that clinicians recognize PADs to be able to provide informed therapeutic options and improve patient outcomes. Here, we summarize the clinical features commonly seen with each of the currently known PADs, we identify clinical warning signs that warrant assessment for PADs, and lastly, we discuss the benefits of timely diagnosis and management of these conditions.
Copyright © 2021. Published by Elsevier Inc.

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