Hyper IgE Syndrome

Hyper-IgE Syndrome (Job’s Syndrome) is a genetic alteration that can lead to a chronic skin rash (eczema) and chronic infections, especially in the skin and lung.

IgE, or Immunoglobulin E, is an antibody that helps protect our body from certain infections. IgE is also made by the body during allergic reactions.

IgE can lead to allergic symptoms of:

  • Coughing, wheezing
  • Runny nose
  • Eye or throat irritation
  • Dry, red and itchy skin

Inheritance of Hyper-IgE Syndrome can be autosomal dominant, or recessive.

Patients with Hyper-IgE have trouble fighting harmful bacteria. The patient may develop the following symptoms:

  • Frequent skin infections
  • Dry, itchy skin (eczema)
  • Frequent pneumonia

As a child gets older, watch for:

  • Delay in loosing primary teeth
  • Large cysts in the lungs, called pneumatoceles
  • Poor bone formation
  • Increased broken bones (fractures)
  • Abnormal curvature of the spine (scoliosis)

Chest x-rays or chest CTs are done regularly to look for cysts in the lungs.

While there is no cure for Hyper-IgE syndrome, treatment focuses on stopping the symptoms. Some common treatments are:

  • Antibiotics to treat infections
  • Cream medication for skin rash
  • Some patients may need to take medication every day to prevent infection
  • Some patients may need to get an IV medicine called IVIG. IVIG contains antibodies to fight future infections.