Hyper-IgE syndrome. The history of disease (from Job's syndrome to defect STAT3 gene)

The lecture is devoted to hyper-IgE recurrent infections syndrome and is based both upon the literature survey and numerous personal observations for more than 25 years. Initial description of this disease by Davis et al (1966) under the name «Job's syndrome: recurrent «cold» staphylococcal abscesses» was followed by remarkable rinding of Buckley et al (1972) connecting this disease with extreme hyper-immunoglobulinemia E. Later on the two district forms of the disease were described: an autosomal dominant and autosomal recessive. Recently (2007) the nature of the most typical autosomal dominant form of the syndrome was discovered as mutations in STAT3. The discovery explains multiple disorders seen in this multisystem syndrome.