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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author Mohammad Mahjoubi
Ronak Rashedi
Noosha Samieefar
Fahimeh Abdollahimajd
Nima Rezaei
author_facet Mohammad Mahjoubi
Ronak Rashedi
Noosha Samieefar
Fahimeh Abdollahimajd
Nima Rezaei
author_sort Mohammad Mahjoubi
collection DOAJ
description 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.
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spelling doaj-art-1ac4016f9e7e4ffda9ded8c8d018f9802025-08-20T03:52:24ZengBMCAllergy, Asthma & Clinical Immunology1710-14922025-05-0121111110.1186/s13223-025-00963-6Dermatologic presentations of hyper IgE syndrome in pediatric patientsMohammad Mahjoubi0Ronak Rashedi1Noosha Samieefar2Fahimeh Abdollahimajd3Nima Rezaei4Network of Interdisciplinarity in Neonates and Infants (NINI), Universal Scientific Education and Research Network (USERN)Network of Interdisciplinarity in Neonates and Infants (NINI), Universal Scientific Education and Research Network (USERN)Network of Interdisciplinarity in Neonates and Infants (NINI), Universal Scientific Education and Research Network (USERN)Network of Interdisciplinarity in Neonates and Infants (NINI), Universal Scientific Education and Research Network (USERN)Network of Interdisciplinarity in Neonates and Infants (NINI), Universal Scientific Education and Research Network (USERN)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.https://doi.org/10.1186/s13223-025-00963-6Job syndromeSkin manifestationsPrimary immunodeficiency diseases
spellingShingle Mohammad Mahjoubi
Ronak Rashedi
Noosha Samieefar
Fahimeh Abdollahimajd
Nima Rezaei
Dermatologic presentations of hyper IgE syndrome in pediatric patients
Allergy, Asthma & Clinical Immunology
Job syndrome
Skin manifestations
Primary immunodeficiency diseases
title Dermatologic presentations of hyper IgE syndrome in pediatric patients
title_full Dermatologic presentations of hyper IgE syndrome in pediatric patients
title_fullStr Dermatologic presentations of hyper IgE syndrome in pediatric patients
title_full_unstemmed Dermatologic presentations of hyper IgE syndrome in pediatric patients
title_short Dermatologic presentations of hyper IgE syndrome in pediatric patients
title_sort dermatologic presentations of hyper ige syndrome in pediatric patients
topic Job syndrome
Skin manifestations
Primary immunodeficiency diseases
url https://doi.org/10.1186/s13223-025-00963-6
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AT fahimehabdollahimajd dermatologicpresentationsofhyperigesyndromeinpediatricpatients
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