Primary immune deficiencies with aberrant IgE production.

[omenn syndrome]

IgE antibodies play a central role in the pathogenesis of atopic diseases and in host immunity against parasitic infections . IgE has potent activities on mast cells and basophils . IgE class switching is a very tightly controlled process , and serum IgE levels are very low compared with other immunoglobulin isotypes . Transcription factors that activate or inhibit the IgE gene promoter , as well as T (H )1 and T (H )2 cytokines are important in the regulation of IgE levels . Hyper-IgE syndrome ; Wiskott-Aldrich syndrome ; immunodysregulation , polyendocrinopathy , enteropathy , X-linked (IPEX ); Omenn syndrome ; and atypical complete DiGeorge syndrome are primary immune deficiencies that are associated with elevated serum IgE levels . Increased IgE levels in IPEX , Wiskott-Aldrich syndrome and Omenn syndrome are likely related to increased T (H )2 cytokine production caused by decreased a number or function of CD 4 (+)CD 2 5 (+)forkhead box protein P 3 (+) regulatory T cells . The link between signal transducer and activator of transcription 3 mutations and elevated serum IgE levels in hyper-IgE syndrome is unclear . Insight into IgE regulation provided by the study of primary immune deficiencies with elevated IgE has important implications for allergic diseases .