Hyper-IgE Syndrome

Hyper-IgE syndromes are rare primary immunodeficiencies (e.g. STAT3, DOCK8, PGM3) with eczematous dermatitis, very high IgE, eosinophilia and recurrent skin and lung infections. STAT3 disease often begins as a pustular newborn rash on the face and scalp; DOCK8 brings severe viral infections such as widespread molluscum or eczema herpeticum (Siegfried & Hebert 2015; Napolitano 2022). A high IgE alone does not mean HIES, because IgE is often high in ordinary Atopic Dermatitis (Napolitano 2022).

Connections

• Differential Diagnosis of Toddler Eczema — immunodeficiency red flag, source: Napolitano 2022