Document detail
ID
oai:pubmedcentral.nih.gov:1093...
Topic
Case ReportAuthor
Li, Weifeng Qi, Qiqi Wang, Weipeng Li, DongqinLangue
enEditor
Lippincott Williams & Wilkins
Category
Asia Pacific Allergy
Year
2023
listing date
3/25/2024
Keywords
dupilumab atopic dermatitisAbstract
Hyper-immunoglobulin E syndrome (HIES) is a primary immunodeficiency disease characterized by atopic dermatitis, recurrent skin and lung infections, and significantly elevated serum immunoglobulin E levels.
Autosomal dominant and loss-of-function pathogenic variants in the STAT3 gene are the most common causes of the disease and studies have shown that the presence of IL-4 receptor (IL-4R) is upregulated in patients with dominant-negative mutations in the STAT3 gene expression.
Dupilumab is a monoclonal antibody that targets the IL-4α receptor and improves the symptoms of atopic dermatitis by inhibiting IL-4 and IL-13.
We used dupilumab to treat severe dermatitis in a patient with STAT3-HIES and achieved satisfactory results.
Li, Weifeng,Qi, Qiqi,Wang, Weipeng,Li, Dongqin, 2023, The treatment efficacy of dupilumab in autosomal dominant hyper-immunoglobulin E syndrome with severe atopic dermatitis, Lippincott Williams & Wilkins
