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Hyperimmunoglobulin E syndrome





Hyperimmunoglobulin E syndrome

Alternative Names:

Job syndrome; Hyper IgE syndrome
Symptoms:
  • Persistent skin abscesses and infection
  • Recurrent pus in the sinus
  • Eczema
  • Bone and tooth defects, including fractures and late shedding of baby teeth

Signs and tests:
  • Keratoconjunctivitis
  • Osteomyelitis
  • Kyphoscoliosis
  • Lung abscesses on chest x-ray
  • Recurrent sinus infection


Tests to confirm a diagnosis:

  • Quantitative immunoglobulins reveal highblood 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:




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