Background Atopic dermatitis (AD) affects up to 25% of children and 10% of adults in
Western countries. When severe or recurrent infections and exceedingly elevated serum IgE …

The caspase activation and recruitment domain 11 (CARD11) gene encodes a scaffold
protein required for lymphocyte antigen receptor signaling. Dominant-negative, loss-of …

The CARD11/BCL10/MALT1 (CBM) complex is a critical signalosome in lymphocytes that
facilitates several downstream immune responses, predominantly through activation of the …

CARD11 is a multidomain scaffold protein required for normal activation of NF-κB, JNK, and
mTOR during Ag receptor signaling. Germline CARD11 mutations cause at least three types …

The Hyper IgE Syndromes are rare primary immunodeficiencies characterized by eczema,
recurrent skin and respiratory infections and elevated serum IgE levels. Nowadays a …

Autosomal dominant hyper-IgE syndrome (AD-HIES), characterised by eczema, increased
susceptibility to skin and lung infections, elevated IgE and skeletal abnormalities is …

Abstract Background Hyper‐IgE syndromes (HIES) are a clinically overlapping,
heterogeneous group of inborn errors of immunity characterized by elevated serum IgE …

Hyper-IgE syndromes and chronic mucocutaneous candidiasis constitute rare primary
immunodeficiency syndromes with an overlapping clinical phenotype. In recent years, a …

Severe allergic disease is common, and few monogenic causes of atopy have been
described. A new study that convincingly links severe atopic dermatitis to heterozygous …

In hyper-IgE syndromes (HIES), a group of primary immunodeficiencies clinically
overlapping with atopic dermatitis, early diagnosis is crucial to initiate appropriate therapy …