The molecular basis of this clinical presentation was first demonstrated to be associated with autosomal dominant-negative mutations in the signal transducer and activator of transcription 3 gene (STAT3), leading to impaired STAT3 signaling, as Job Syndrome has been redefined as STAT3 dominant-negative hyper-IgE syndrome (STAT3 HIES). The gene discussed is STAT3; the disease is hyper-IgE syndrome.