Chronic rhinosinusitis with severe nasal polyposis in Hyper-IgE syndrome

Autosomal-dominant Hyper-IgE syndrome (AD-HIES) is a rare multisystem disorder that is characterized by eczema, infections with staphylococcal and fungal organisms, elevated total serum IgE level and skeletal and connective tissue involvement. The transcription factor signal transducer and activator of transcription 3 (STAT3) plays a crucial role in the development and differentiation of Th17 cells and mutations in STAT3 cause AD-HIES.1 Patients with AD-HIES are reported to have markedly decreased or absent circulating Th17 cells.

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