The Journal of allergy and clinical immunology, vol.125, no.2, 2010 (Peer-Reviewed Journal)
Article / Article
The Journal of allergy and clinical immunology
Science Citation Index Expanded, Scopus
Hyper-IgE syndrome, HIES, Job syndrome, T(H)17 cells, STAT3 mutations, diagnostic guidelines, HOST-DEFENSE, CELLS
Background: The hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by infections of the lung and skin, elevated serum IgE, and involvement of the soft and bony tissues. Recently, HIES has been associated with heterozygous dominant-negative mutations in the signal transducer and activator of transcription 3 (STAT-3) and severe reductions of T(H)17 cells.