Dermatologic presentations of hyper IgE syndrome in pediatric patients

Abstract Background Hyper-IgE Syndrome, also known as Job’s syndrome, is a rare primary immunodeficiency disorder characterized by recurrent infections and elevated levels of immunoglobulin E. While respiratory and systemic manifestations have been more emphasized, dermatological manifestations in H...

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Main Authors: Mohammad Mahjoubi, Ronak Rashedi, Noosha Samieefar, Fahimeh Abdollahimajd, Nima Rezaei
Format: Article
Language:English
Published: BMC 2025-05-01
Series:Allergy, Asthma & Clinical Immunology
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Online Access:https://doi.org/10.1186/s13223-025-00963-6
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Summary:Abstract Background Hyper-IgE Syndrome, also known as Job’s syndrome, is a rare primary immunodeficiency disorder characterized by recurrent infections and elevated levels of immunoglobulin E. While respiratory and systemic manifestations have been more emphasized, dermatological manifestations in Hyper-IgE Syndrome also play a significant role in disease presentation. Methods This narrative review explores the dermatologic presentations of Hyper-IgE Syndrome in pediatric populations, including descriptions, associated symptoms/findings, and available treatment options. Results and conclusion Neonatal rash, mucocutaneous candidiasis, noma neonatorum, psoriasis, cold staphylococcal abscesses, and candida onychomycosis are among the dermatological manifestations of Hyper-IgE Syndrome. Each manifestation has unique characteristics and treatment considerations, necessitating accurate recognition and diagnosis for effective management. Optimal treatment strategies involve a combination of supportive care, topical/systemic therapies, antifungal medications, and surgical interventions when necessary. Further research is needed to enhance our understanding of these manifestations and evaluate treatment modalities for individuals affected by Hyper-IgE Syndrome.
ISSN:1710-1492