title: [Hyper IgE syndrome: three case reports].
abstract: BACKGROUND
  Autosomal dominant Hyper IgE syndrome (HIES-AD) is a primary immunodeficiency associated with connective tissue, skeletal, vascular and brain disorders. The pathogenesis of immune deficiency lies in an alteration of Th17 cells which explains the special susceptibility of these patients to S. aureus and Candida infections.
  
  
  OBJECTIVE
  To describe three children diagnosed with hyper IgE syndrome and conduct a study on the subject, with special focus on the dominant form of the disease.
  
  
  METHODS
  3 children with HIES-AD (2 males and one female) with eczema since birth, skin, ear, lung, and lymph node infections, and serum IgE levels over 2,000 IU/ml and eosinophilia values, treated with antibiotics and topically, and 7 year follow-up.
  
  
  CONCLUSIONS
  It is a rare condition that requires a high index of suspicion and early management of infections. One of its main diagnoses is atopic syndrome with recurrent infections but both conditions differ in context, response and resolution against infections and lack of other phenotypic characteristics.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Tagle C
authors.forename: Alvaro
authors.initials: A
authors.lastname: Melys G
authors.forename: Angela
authors.initials: A
authors.lastname: Castillo M
authors.forename: Ximena
authors.initials: X
authors.lastname: Norambuena R
authors.forename: Arnoldo
authors.initials: A
authors.lastname: Quezada L
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004485:Eczema / Q000209:etiology* / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood*; D007223:Infant; D007589:Job Syndrome / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D008297:Male
chemical_list: D007073:Immunoglobulin E