Hyper IgE Syndrome

What is Hyper IgE Syndrome?

Hyper IgE Syndrome (HEIS) is a rare primary immunodeficiency disease.

About Dr José Costa

Dr José Costa (MD, FRCPCH, PGCert Allergy, PGCert Paed Allergy) is a Consultant Paediatrician and fellow of the Royal College of Paediatrics and Child Health.
He has over 14 years' experience in Paediatrics and over 7 years in Paediatric Allergy.

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Hyper IgE Syndrome Symptoms

Some of the most common symptoms of HIES include:

• eczema
• recurrent skin abscesses
• recurrent pneumonia​
• high serum levels of IgE
  
• increased number of eosinophils
  

It can also affect the bones, teeth and connective tissue.

Hyper IgE Syndrome Diagnosis

HIES is best diagnosed by a combination of clinical findings and blood tests. Sometimes CTs and MRIs need to be done as well.
In almost every patient we would expect to see an elevated level of serum IgE. However, as it is possible to see the same IgE levels in patients suffering with severe eczema.
We combine these findings with other tests like genetic testing which can identify gene mutations, which can confirm the diagnosis.

Hyper IgE Syndrome Treatment

Treatment of hyper-IgE syndrome will vary according to the presenting symptoms.

• Eczema - prophylactic antibiotics, moisturizers, steroids, tacrolimus
  
• Lungs - antibiotics
  
• Fingernails, mouth or vagina - antifungals
  

For more severe abscesses, there might be needed surgical intervention.