Hyperimmunoglobulin E syndrome

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Job syndrome; Hyper IgE syndrome

• Persistent skin abscesses and infection
• Recurrent pus in the sinus
• Eczema
• Bone and tooth defects, including fractures and late shedding of baby teeth

• Keratoconjunctivitis
• Osteomyelitis
• Kyphoscoliosis
• Lung abscesses on chest x-ray
• Recurrent sinus infection

Tests to confirm a diagnosis:

• Quantitative immunoglobulins reveal high blood IgE levels
• Marked eosinophilia  (large amount of white blood cells) by CBC with differential
• Defective neutrophil and monocyte chemotactic studies (though not in all cases)
• Absent delayed hypersensitivity (DCNB, candidal)

Other tests may be done in response to infections:

• X-ray of the sinuses
• X-ray of the bones
• X-ray and CT scan of the chest
• Cultures of infected site

Review Date: 08/03/2005
Reviewed By: Frederic F. Little, M.D., Department of Allergy and Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston, MA. Review provided by VeriMed Healthcare Network.