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Autosomal dominant hyper-IgE syndrome

What is Autosomal dominant hyper-IgE syndrome?

A very rare primary immunodeficiency disorder characterized by the clinical triad of high serum IgE (>2000 IU/ml), recurring staphylococcal skin abscesses, and recurrent pneumonia with formation of pneumatoceles.

Other condition names

  • AD-HIES
  • Autosomal dominant HIES
  • Autosomal dominant hyperimmunoglobulin E syndrome
  • Buckley syndrome
  • Hyperimmunoglobulin E syndrome type 1
  • Hyperimmunoglobulin E-recurrent infection syndrome
  • Job syndrome
  • STAT3 deficiency

Inheritance type

Autosomal dominant

Prevalence

  • Europe: 1-9 in 100,000

Age of Onset

  • Infancy
  • Neonatal
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.