IGHE and hyper-IgE syndrome: Hyperimmunoglobulin E syndromes (HIES) were originally defined as two primary variants, each with mutations in a different gene: an autosomal dominant variant caused by loss of function of the signal transducer and activator of transcription (STAT) 3 gene, STAT3, and an autosomal recessive variant due to loss of function of the dedicator of cytokinesis 8 gene, DOCK8. Over time, numerous genotypes with similar clinical phenotypes have been identified; HIES has thus become somewhat of a misnomer as many PADs may present with very elevated IgE levels (25, 26).