People with HIES have recurrent staphylococcal abscesses (boils) of the skin and lungs. They also have an itchy skin rash (eczema), very high levels of a type of antibody called immunoglobulin E (IgE), and a tendency to have bone fractures.
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Causes
Signs and symptoms
People with HIES may have repeated bacterial abscesses of the skin and lungs. These infections are often caused by Staphylococcus aureus, but also may be caused by fungal organisms. They also have recurrent bone fractures and severe itching and inflamed skin. They may keep their baby teeth.
People with HIES often have distinctive facial characteristics, such as the following:
- Uneven facial features
- Prominent forehead
- Deep-set eyes
- Broad nasal bridge
- Wide, fleshy nose tip
- Protruding lower jaw
Read about the immune cells missing in people with HIES.
Diagnosis
A doctor will suspect HIES in a person with recurrent staphylococcal abscesses and itchy skin rashes and recurring staph infections or pneumonias.
Blood tests diagnosing HIES will show normal levels of IgG, IgA, and IgM antibodies but very high levels of IgE antibodies. They also may show a high number of white blood cells called eosinophils and a poor response to immunizations.
Treatment
The most effective treatment for HIES is continuous anti-staphylococcal antibiotics and antifungals as needed.