The hyper-IgE syndromes (HIES) are a heterogeneous group of inborn errors of immunity
sharing manifestations including increased infection susceptibility, eczema, and raised …

In addition to susceptibility to infections, conventional primary immunodeficiency disorders
(PIDs) and inborn errors of immunity (IEI) can cause immune dysregulation, manifesting as …

Dupilumab was first approved for the treatment of atopic dermatitis (AD) and blocks the
signaling of interleukin (IL)-4 and-13. Several other chronic skin conditions share …

Around 20% of all children worldwide suffer from atopic dermatitis. Therefore, eczematous
skin lesions and elevated serum immunoglobulin E (IgE) levels are common findings. Inborn …

Background Cutaneous manifestations of dedicator of cytokinesis 8 gene (DOCK8)
deficiency, a combined type of T and B cell immunodeficiency, previously designated as …

Atopic dermatitis (AD) is one of the most common skin conditions in humans. AD affects up
to 20% of children worldwide and results in morbidity for both patients and their caregivers …

Dupilumab interferes with the signaling pathways of IL‐4 and IL‐13 and is effective in
treating atopic dermatitis. Specific genodermatoses, including Netherton syndrome …

Highlights•Heterozygous mutations in ERBIN disrupt regulation of TGFb signaling via
STAT3.•Loss of ERBIN function links the atopic and connective tissue abnormalities seen in …

Relieving job: Dupilumab in autosomal dominant STAT3 hyper-IgE syndrome. - Abstract -
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Background Autosomal dominant hyper-IgE syndrome (AD-HIES) caused by dominant
negative (DN) variants in the signal transducer and activator of transcription 3 gene (STAT3) …