Pathogenesis of hyper IgE syndrome.

  title={Pathogenesis of hyper IgE syndrome.},
  author={Jennifer Heimall and Alexandra F Freeman and Steven M Holland},
  journal={Clinical reviews in allergy & immunology},
  volume={38 1},
Hyper IgE syndrome (HIES) is a rare primary immunodeficiency characterized by the triad of elevated IgE and eosinophilia, eczema, and recurrent skin and pulmonary infections. The autosomal dominant (AD) form of HIES results from mutations in STAT3 and is characterized by disordered inflammation, connective tissue, and skeletal abnormalities. Tissue-specific STAT3 deficiency in animals, cytokine and transcriptional array data, and careful clinical phenotyping have explained some of the… CONTINUE READING
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