Article
Clearance of atypical cutaneous manifestations of hyper-IgE syndrome with dupilumab.
Pediatric dermatology - 1 Nov 2022
Nihal Aman, Comstock Jeanette R, Holland Kristen E, Singh Anne Marie, Seroogy Christine M, Arkin Lisa M
Abstract excerpt
Hyper-IgE syndromes (HIES) are a heterogeneous group of rare primary immunodeficiency diseases classically characterized by the triad of atopic dermatitis, and recurrent cutaneous and pulmonary infections. Autosomal dominant, loss-of-function STAT3 pathogenic variants are the most common genetic cause, which lead to deficiency of Th17 lymphocytes, impaired interferon gamma production, and IL-10 signal...
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