Severe dermatitis in a 24-day-old baby with hyper-IgE syndrome

Hyper-immunoglobulin E syndromes (HIES), also known as Job's syndrome, is a rare primary immunodeficiency disorder characterized by elevated serum IgE levels, recurrent cutaneous and sinopulmonary infections, and chronic eczematous dermatitis. The condition can manifest in two familial forms: autosomal dominant (AD-HIES) and autosomal recessive (AR-HIES), with most cases being sporadic. Herein we present the case of a patient with HIES associated with a mutation in the signal transducer and activator of transcription 3 (STAT3) gene. This case highlights the importance of recognizing the clinical manifestations of HIES in neonates. Early diagnosis through clinical evaluation and genetic testing is crucial for effective management and improving the quality of life for affected individuals.