Related Experiment Video
Updated: Aug 15, 2026
Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
Severely impaired IL-12/IL-18/IFNgamma axis in patients with hyper IgE syndrome
M G Netea1, B J Kullberg, J W M van der Meer
1Radboud University Medical Centre and Nijmegen University Center for Infectious Diseases, Nijmegen, the Netherlands.
Background:
Hyperimmunoglobulin E syndrome (HIES) is an inborn disorder characterized by recurrent skin and respiratory tract infections, skeletal abnormalities, chronic eczema and elevated serum IgE. The nature of the host defence defect has yet to be established. The aim of this study was to investigate whether activation of the interleukin-12/interleukin-18/interferon-gamma axis, known to be crucial for the activation of cellular immune responses, is impaired in patients with HIES.
Materials And Methods:
Cytokine production capacity of seven HIES patients and seven healthy controls was investigated in whole-blood cultures stimulated with heat-killed Staphylococcus aureus, Candida albicans, or a combination of interleukin (IL)-12/IL-18.
Results:
Interferon (IFN)gamma production, in addition to IFNgamma/IL-10 ratios, was 10-30-fold lower in the HIES patients compared with the healthy volunteers. In contrast TNF, IL-1beta and IL-8 production was normal.
Conclusions:
These data revealed a severe dysbalance towards a Th2 phenotype in HIES patients which is likely to contribute to the specific pattern of infection susceptibility characteristic to HIES.
