Job's Syndrome (Hyper IgE syndrome)
also see Phagocyte dysfunction, general

• Problem:
  • hyper IgE
  • problems with: phagocyte chemotaxis and T-cell regulation (normal phagocytes)
  • relatively rare primary immunodeficiency
• Inheritance: autosomal dominant with variable expression
• Presentation:
  • Recurrent sinopulmonary infections and skin abscesses (severe staphylococcal abcesses, pneumatocele formation)
  • pruritic dermatitis; eczema starting as neonate
  • coarse facies and red hair
  • Can get pneumatoceles
  • delayed shedding of primary dentition
  • frequent bone fractures
• Labs: Very elevated IgE, pronounced eosinophilia in blood and sputum
• Rx: Supportive care,  Prophylactic penicillinase-resistant Abx, prophylactic TMP-SMX, other Abx prn (anti-staph),  +/-IVIG

CHLA Board review 2005