ArticleFrontiers in immunology2026
Case Report: Effective IL-4/IL-13 axis suppression and resolution of severe atopic dermatitis by dupilumab monotherapy in a patient with IPEX syndrome.
Article in Frontiers in immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: The treatment of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome has largely focused on immunosuppression with little impact on the atopic manifestations of the disease. Dupilumab therapy has been used successfully in primary atopic diseases and immune regulatory disorders to control type 2 inflammation. Indeed, it has been reported in two prior IPEX cases, but in combination with immunosuppressive agents. Here, we are the first to report the clinical and immunologic impact of dupilumab monotherapy in IPEX. Case: A 4-year-old boy with a confirmed hemizygous Forkhead box protein 3 (FOXP3) missense variant (c.1150G>A; p.Ala384Thr) presented with severe, treatment-refractory atopic dermatitis beginning in the newborn period, failure to thrive, lymphadenopathy, food and environmental allergies, asthma, recurrent febrile neutropenia, and type 1 diabetes mellitus, with a peak IgE of 74,625 IU/mL. Following initiation of dupilumab monotherapy at age 2, the patient had a marked improvement in atopic dermatitis by Eczema Area and Severity Index (EASI) score, a rapid decline in serum IgE, improved growth, reduced respiratory hospitalizations, and a profoundly improved quality of life. Results: Immunologically, dupilumab effectively suppressed IgE and plasma interleukin-4 (IL-4) and IL-13 to healthy control levels. In addition, C-C motif chemokine ligand 27 (CCL27), important for cutaneous T-cell trafficking, was detected at healthy control levels. Upstream alarmins, eosinophil mediators, T helper 1 (Th1) cytokines, and Treg-specific demethylated region (TSDR) demethylation remained elevated-indicating ongoing systemic immune dysregulation not fully addressed by dupilumab alone. Conclusion: This case demonstrates that dupilumab monotherapy can effectively suppress the IL-4/IL-13 axis and substantially improve atopic manifestations in IPEX, potentially by restoring skin-specific immune tolerance through rebalancing cutaneous T-cell trafficking. These findings support dupilumab as a targeted adjunctive therapy for the atopic features of IPEX and provide a rationale for the systematic study of dupilumab alone or in combination with other immunosuppression regimens.
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