Use of dupilumab for atopic dermatitis in pediatric and young adult patients with inborn errors of immunity

Abstract

To the Editor, Inborn errors of immunity (IEIs) are inherited disorders characterized by defects in both innate and adaptive immunity. More than 450 distinct defects are described in the 2022 classification of the International Union of Immunological Societies (IUIS). 1 IEIs present with a variety of symptoms. The hallmark is recurrent and severe infections often treated with intravenous and/or prolonged antibiotic therapy. Patients with IEIs also commonly experience immune dysregulation manifestations such as autoimmune and inflammatory diseases, and increased susceptibility to cancer and atopy. 2 Among atopic manifestations dermatitis, asthma, and food allergies are the most frequently observed.Atopic dermatitis (AD) in IEIs typically manifests with early onset, a recurrent or relapsing course, and a poor response to conventional medical therapy. Dermatitis is characterized by intense itching, dry skin, erythematous lesions, erosions, and exudation. In the chronic phase, lichenification may occur.Preserving the integrity of the skin barrier is a key goal in AD therapy. The first-line treatment involves topical therapy such as emollients and corticosteroid anti-inflammatory drugs.