Short answer · Medically reviewed summary · Last updated: 2026-04-07

Hyper IgE Syndrome (HIES), often referred to as Job’s syndrome, is characterized by a triad of recurrent skin abscesses, pneumonia, and significantly elevated levels of serum immunoglobulin E (IgE). Clinical Presentation and Early Warning Signs In patients with Hyper IgE Syndrome, the clinical presentation is multisystemic. The most common hallmark is the development of "cold" staphylococcal skin abscesses—so named because they lack the typical redness, warmth, and pain associated with standard infections.

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Which are the symptoms of Hyper IgE Syndrome?

Symptoms of Hyper IgE Syndrome reported by real patients, from the most common to the most limiting, plus a medically reviewed summary with sources.

Hyper IgE Syndrome symptoms

Hyper IgE Syndrome (HIES), often referred to as Job’s syndrome, is characterized by a triad of recurrent skin abscesses, pneumonia, and significantly elevated levels of serum immunoglobulin E (IgE).



Clinical Presentation and Early Warning Signs


In patients with Hyper IgE Syndrome, the clinical presentation is multisystemic. The most common hallmark is the development of "cold" staphylococcal skin abscesses—so named because they lack the typical redness, warmth, and pain associated with standard infections. Early warning signs for caregivers to watch for include severe eczema starting in early infancy, persistent fungal infections (such as thrush), and recurrent bacterial pneumonias that may lead to the formation of pneumatoceles (air-filled cysts in the lungs).



Symptom Variability and Daily Impact


Symptoms of Hyper IgE Syndrome vary significantly between patients, even within the same family. Some individuals may experience primarily dermatological issues, while others suffer profound skeletal abnormalities, such as retained primary teeth, scoliosis, or bone fractures due to decreased bone density. Daily quality of life is most often impacted by the constant vigilance required to manage chronic skin inflammation and the psychological burden of recurring respiratory infections that necessitate frequent hospitalizations.



Progression and Emergency Care


Over time, the chronic inflammatory nature of Hyper IgE Syndrome can lead to structural changes in the lungs, such as bronchiectasis. While management has improved, the disease remains complex throughout the lifespan. You must seek immediate medical attention if you notice signs of systemic infection, such as high fever, sudden difficulty breathing, or the rapid spread of skin lesions, as these can progress quickly in individuals with this immune deficiency.



Disclaimer: This information is for educational purposes and does not substitute for professional medical advice, diagnosis, or treatment. Always consult with your immunologist or primary healthcare provider regarding specific symptoms related to Hyper IgE Syndrome.



References



  • NIH Genetic and Rare Diseases Information Center (GARD): Hyper IgE syndrome

  • Orphanet: Autosomal dominant hyper-IgE syndrome

  • Online Mendelian Inheritance in Man (OMIM): Hyper-IgE recurrent infection syndrome (HIES)

Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2026-04-07
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
2 answers
Translated from portuguese Improve translation
• - growth deficit
• Teething problems
• Hyper-extensibility joints
• Ichthyosis
• -Asthma
• -Rhinitis
• -Sinusitis
• -Otitis
• Scoliosis
• -Hodgkin's lymphoma
• -Candidiasis
• Infections of the bladder
• Eosinophilia
• -Herpes
• facial features coarse
• -Appearance of coarse facial .

Causes of secondary caused by medications can appear as:
• Osteogenia
• Cushing's syndrome
• Diabetes
• Esophagitis
• Stretch marks
• Abdominal fat is located

Posted May 8, 2017 by Gina Harla 2515

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Stories of Hyper IgE Syndrome

HYPER IGE SYNDROME STORIES
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12 surgeries (3 hernias, 3 sinusitis, 3 esophageal dilations, staff infection, meningitis, degenerative disc)  
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_Procuro pessoas no Mundo especialmente no Brasil com esta doença_ _Meus níveis de igE ja chegaram a 40.000ui, por outro lado tenho infecções recorrentes de Bexiga e não a de pulmão como a maioria, por isso penso ter uma variação da doença ...
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I am adopted - a trans-racial adoptee - and am part of a blended family. I am the only African child, but have three siblings - brother and two sisters. I am the only one with Hyper IgE. I have no knowledge of my biological parents' medical history. ...
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  Nasci prematura e já com quadro de pneumonia logo nos primeiros dias de vida. Já tive mais de 28 pneumonias radiografadas e muitas outras diagnosticas apenas clinicamente. Infeções de pele na infância e adolescência.Quando adulta melhorei d...
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My 12 year old son has Hyper Ige syndrome, Stat 3.  He was diagnosed when he was 5.

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