Short answer · Medically reviewed summary · Last updated: 2026-04-07
Hyper IgE Syndrome (HIES), also known as Job syndrome, is an ultra-rare primary immunodeficiency disorder with an estimated prevalence of approximately 1 in 100,000 to 1 in 1,000,000 individuals worldwide. Epidemiology and Distribution Because Hyper IgE Syndrome is exceptionally rare, precise incidence rates are difficult to calculate, and current estimates are largely based on clinical case series rather than large-scale population screening. The condition affects males and females with equal frequency, showing no clear gender-based disparity.
What is the prevalence of Hyper IgE Syndrome?
Prevalence of Hyper IgE Syndrome: how many people are affected worldwide, differences by sex and region, with sources.
Hyper IgE Syndrome (HIES), also known as Job syndrome, is an ultra-rare primary immunodeficiency disorder with an estimated prevalence of approximately 1 in 100,000 to 1 in 1,000,000 individuals worldwide.
Epidemiology and Distribution
Because Hyper IgE Syndrome is exceptionally rare, precise incidence rates are difficult to calculate, and current estimates are largely based on clinical case series rather than large-scale population screening. The condition affects males and females with equal frequency, showing no clear gender-based disparity. While symptoms of Hyper IgE Syndrome typically manifest during infancy or early childhood—often presenting as recurrent staphylococcal skin abscesses or pneumonias—the age of onset can vary, and some patients may not receive a formal diagnosis until adulthood.
Geographic and Diagnostic Challenges
There is no evidence suggesting that Hyper IgE Syndrome is more common in specific geographic regions or ethnic groups; however, the disorder is frequently underdiagnosed or misdiagnosed as severe eczema or chronic infections. This diagnostic delay significantly complicates our ability to track true prevalence. At DiseaseMaps.org, we have seen 134 individuals join our community to share their experiences, providing a vital, real-world perspective that complements clinical data. These patient-reported experiences highlight that the global burden of Hyper IgE Syndrome may be higher than currently recorded in medical literature due to these gaps in diagnostic awareness.
Clinical Perspective
For those navigating Hyper IgE Syndrome, it is important to remember that these statistical estimates are snapshots in time. As genetic sequencing becomes more accessible, we expect a more nuanced understanding of the prevalence of various subtypes, such as those associated with mutations in the STAT3 or DOCK8 genes. Our community at DiseaseMaps.org continues to serve as a hub for those seeking to connect with others who truly understand the daily realities of managing this rare condition.
Medical Disclaimer: This information is for educational purposes and should not replace professional medical advice, diagnosis, or treatment. Always consult with your immunologist or healthcare provider regarding your specific health situation.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Hyper IgE Syndrome
- Orphanet: Autosomal dominant hyper-IgE syndrome
- OMIM (Online Mendelian Inheritance in Man): Hyper-IgE recurrent infection syndrome
