A Clinical Conundrum in Patients With a Common Clinical Spectrum but Rare Differentials: Hyper-Immunoglobulin E Syndrome and Idiopathic Eosinophilia

Hyper-immunoglobulin E syndrome (HIES) and idiopathic eosinophilia lie on the same spectrum of immunodeficiency disease; therefore, physicians need to consider both possibilities as differential diagnoses for early identification. HIES is a primary immunodeficiency syndrome characterized by markedly elevated total IgE levels along with recurrent eczematous rash, skin infections, sinopulmonary infections, and multiple hospitalizations. Similarly, patients with idiopathic hypereosinophilic syndrome (HES) also present with marked eosinophilia, recurrent sinopulmonary infections, and evidence of organ damage such as eosinophilic myositis. Idiopathic eosinophilia also lacks any identifiable secondary aetiology and evidence of organ damage, with only eosinophilia in bone marrow. Both our cases have similar symptoms, presenting in the busy outpatient department settings that needed thoughtful consideration for the differentials. Awareness among Clinicians about this wide spectrum of differentials with overlapping presentations will facilitate early diagnosis and eventually enable optimal cost-effective treatment for patients.

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Publication Details

Journal
Cureus
Published
2025-08-12
DOI
https://doi.org/10.7759/cureus.89898
Primary Topic
Eosinophilic Disorders and Syndromes
Type
article
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0.00
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article

A Clinical Conundrum in Patients With a Common Clinical Spectrum but Rare Differentials: Hyper-Immunoglobulin E Syndrome and Idiopathic Eosinophilia

Adesh Kumar, Somnath Bhattacharya, Prashant Kumar Yadav
Cureus
Eosinophilic Disorders and Syndromes
article

A Clinical Conundrum in Patients With a Common Clinical Spectrum but Rare Differentials: Hyper-Immunoglobulin E Syndrome and Idiopathic Eosinophilia

Adesh Kumar, Somnath Bhattacharya, Prashant Kumar Yadav
article en

Abstract

Hyper-immunoglobulin E syndrome (HIES) and idiopathic eosinophilia lie on the same spectrum of immunodeficiency disease; therefore, physicians need to consider both possibilities as differential diagnoses for early identification. HIES is a primary immunodeficiency syndrome characterized by markedly elevated total IgE levels along with recurrent eczematous rash, skin infections, sinopulmonary infections, and multiple hospitalizations. Similarly, patients with idiopathic hypereosinophilic syndrome (HES) also present with marked eosinophilia, recurrent sinopulmonary infections, and evidence of organ damage such as eosinophilic myositis. Idiopathic eosinophilia also lacks any identifiable secondary aetiology and evidence of organ damage, with only eosinophilia in bone marrow. Both our cases have similar symptoms, presenting in the busy outpatient department settings that needed thoughtful consideration for the differentials. Awareness among Clinicians about this wide spectrum of differentials with overlapping presentations will facilitate early diagnosis and eventually enable optimal cost-effective treatment for patients.

CureusVol. 17(8)
Adesh University (IN)
Openalex Percentile: Top 68%
Eosinophilic Disorders and Syndromes
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A Clinical Conundrum in Patients With a Common Clinical Spectrum but Rare Differentials: Hyper-Immunoglobulin E Syndrome and Idiopathic Eosinophilia — Adesh Kumar, Somnath Bhattacharya, et al. · Cureus (2025) | TGRS Research Map | TGRS