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Dermatologic Presentations of Hyper Ige Syndrome in Pediatric Patients Publisher



Mahjoubi M1, 2 ; Rashedi R1 ; Samieefar N1, 3 ; Abdollahimajd F1, 4, 5 ; Rezaei N1, 6, 7
Authors
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Authors Affiliations
  1. 1. Network of Interdisciplinarity in Neonates and Infants (NINI), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  2. 2. Clinical Research Development Center, Najafabad Branch, Islamic Azad University, Najafabad, Iran
  3. 3. Pediatric Chronic Kidney Disease Research Center, Gene, Cell & Tissue Research Institute, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Clinical Research Development Unit, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  5. 5. Research Center of Artificial Intelligence in Health, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  6. 6. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Allergy, Asthma and Clinical Immunology Published:2025


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. © The Author(s) 2025.
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