Health condition
Hyper-IgE syndrome
Hyper-IgE syndrome (Job syndrome) - classically from dominant-negative STAT3 mutations - combines eczema, recurrent 'cold' skin abscesses and lung infections with distinctive connective tissue features: retained baby teeth, fractures, a characteristic face and dramatically raised IgE.
Why trust this guide
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Editorial team
Written by our editorial team from the UK sources listed on each page.
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Sourced from guidance
Checked against current NHS and UK specialist society guidance at the time of review.
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Current for 2026
Reviewed on 2026-09-27 with the next review scheduled for 2027-09-27.
Key facts
The essentials at a glance.
The symptoms to recognise, when to seek help, which specialist to see, and what treatment usually involves.
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The classic triad: severe eczema from infancy, recurrent staphylococcal skin abscesses (often 'cold' - lacking redness and pain), and recurrent pneumonias
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The give-away complication: pneumatoceles - air-filled cavities left behind after pneumonia - which invite aspergillus infection
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Non-immune clues: retained primary teeth, hyperextensible joints, fractures from minor trauma, scoliosis and characteristic facial features
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IgE is typically very high, but the diagnosis is clinical and genetic (STAT3 and related genes), scored with the NIH system
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Management: continuous anti-staphylococcal prophylaxis, antifungal vigilance, skin care, and careful surgical decisions about lung cavities
Understanding the condition
What it is and what happens next.
What causes it, how it is diagnosed, and the treatment routes available in the UK.
What it is and how it feels
STAT3 sits at a crossroads of immune signalling and tissue biology, which is why one mutation produces such an unlikely portfolio: Th17 responses fail, so staphylococcus and candida thrive at skin and lung; inflammation signals misfire, so abscesses form without heat or pain and pneumonias grumble without fever; and connective tissue develops differently, keeping baby teeth in place, loosening joints and weakening bone. Patients often collect diagnoses - eczema, asthma, brittle bones - for years before anyone adds them up.
Diagnosis and treatment routes
The NIH scoring system formalises the addition, and genetic testing confirms it, distinguishing STAT3 disease from DOCK8 deficiency and other high-IgE conditions that are managed differently. Long-term care is preventative: daily anti-staphylococcal antibiotics, bleach baths or antiseptic skin regimens, aggressive treatment of pneumonias with follow-up imaging for pneumatoceles, antifungals when cavities colonise, and dental and orthopaedic input. This is a lifelong multi-system condition needing an immunology home; private specialist review is typically available within about two weeks.
Seeing a specialist
Who to see, and how quickly you can be seen.
The specialist for this condition is usually a consultant clinical immunologist. Your GP can refer you on the NHS, or you can arrange a private consultation directly.
A quiet reminder
This guide is for information, not medical advice.
Your GP knows your history and can tell you which parts apply to you. If in doubt, see them - and seek urgent care for any of the emergency symptoms described above.
Frequently asked
Common questions about Hyper-IgE syndrome.
Quick, plain-English answers to what people ask us most.
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Can you explain what Hyper-IgE syndrome is?
Hyper-IgE syndrome describes a recognised clinical condition that a doctor can diagnose based on your symptoms, history, and any relevant tests. Its impact varies from person to person, so a proper assessment is the best way to understand your own case. Your GP or a specialist can talk you through what it means in practice.
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Why does hyper-IgE syndrome happen?
Hyper-IgE syndrome can develop for a number of reasons, and the exact cause often depends on individual factors such as underlying health, lifestyle, or in some cases injury. A clinician will usually take a history and may arrange tests to identify what's driving it in your case. Understanding the underlying cause helps guide the most appropriate treatment.
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What are the symptoms of hyper-IgE syndrome?
The presentation of hyper-IgE syndrome differs between individuals, but it typically involves noticeable changes that prompt people to seek medical advice. Some people have mild symptoms that come and go, while others experience more persistent or severe effects. A clinical assessment is the most reliable way to confirm what's going on.
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Is hyper-IgE syndrome serious or dangerous?
Severity varies considerably, and while many cases are manageable with appropriate care, some can be more serious and need prompt attention. It's best not to self-diagnose severity, as similar symptoms can have very different underlying causes. A clinician can assess your specific situation and advise on the right next steps.
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Does hyper-IgE syndrome run in families?
There can be a hereditary element for some people, though many cases occur without any clear family link. If a close relative has been diagnosed with something similar, it's worth mentioning this to your doctor. This can help inform whether any additional monitoring or testing is worthwhile.
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Can hyper-IgE syndrome be treated or reversed?
Many cases can be effectively managed or improved with the right treatment, although whether it can be fully "cured" depends on the underlying cause. Early treatment generally gives the best chance of a good outcome. Your clinician can explain what's realistic to aim for in your specific case.
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What treatment options are available for hyper-IgE syndrome?
Depending on the cause and severity, treatment might involve medication, targeted therapy, monitoring, or in some cases a procedure. Most treatment plans start with the least invasive appropriate option and escalate only if needed. Your care team will personalise this based on your assessment and preferences.
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How long does hyper-IgE syndrome last?
Duration varies significantly depending on the cause, how promptly it's treated, and individual factors, so it's hard to give a single answer that applies to everyone. Some cases resolve within weeks with treatment, while others need longer-term management. Your clinician can give you a more specific timeframe once they understand your case fully.
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When would I need to see a specialist for hyper-IgE syndrome?
If first-line treatment isn't giving enough improvement, or if there are features that suggest a more complex underlying problem, a specialist referral is usually the next step. More invasive treatment is typically considered only after simpler options have been tried and haven't worked well enough. A specialist can assess whether this applies to your situation.
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When should I see a doctor about hyper-IgE syndrome?
It's worth getting checked if things aren't improving, are getting worse, or are causing you ongoing worry or disruption. On the NHS, routine referrals for this kind of assessment can take 20-45+ weeks to come through; going private typically means being seen by a consultant within about 2 weeks.