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Review
. 2025 May 2;21(1):20.
doi: 10.1186/s13223-025-00963-6.

Dermatologic presentations of hyper IgE syndrome in pediatric patients

Mohammad Mahjoubi  1   2 , Ronak Rashedi  1 , Noosha Samieefar  1   3 , Fahimeh Abdollahimajd  1   4   5 , Nima Rezaei  6   7   8
Affiliations
Review

Dermatologic presentations of hyper IgE syndrome in pediatric patients

Mohammad Mahjoubi et al. Allergy Asthma Clin Immunol. .

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.

Keywords: Job syndrome; Primary immunodeficiency diseases; Skin manifestations.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: Ethical approval is not applicable to review studies. Informed consent was obtained from the patient for the picture. Consent for publication: Informed consent was obtained from the patient for the picture publication. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Generalized erythematous and excoriated papules suggesting an eczematous dermatitis in a 10-year-old boy with HIES
See this image and copyright information in PMC

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