Short answer · Medically reviewed summary · Last updated: 2026-04-07
Hyper IgE Syndrome is most commonly referred to as Job Syndrome or Buckley Syndrome, though it is officially classified in medical literature as Autosomal Dominant Hyper-IgE Syndrome (AD-HIES) or Autosomal Recessive Hyper-IgE Syndrome (AR-HIES) depending on the genetic mutation. Historical and Alternative Names The term Job Syndrome was coined in 1966, referencing the biblical figure Job, who was afflicted with "loathsome sores" from head to toe, mirroring the severe recurrent skin abscesses often seen in patients. Another historical name, Buckley Syndrome, honors Dr.
Hyper IgE Syndrome synonyms
Other names for Hyper IgE Syndrome: synonyms, acronyms and related terms used by doctors and patients.
Hyper IgE Syndrome is most commonly referred to as Job Syndrome or Buckley Syndrome, though it is officially classified in medical literature as Autosomal Dominant Hyper-IgE Syndrome (AD-HIES) or Autosomal Recessive Hyper-IgE Syndrome (AR-HIES) depending on the genetic mutation.
Historical and Alternative Names
The term Job Syndrome was coined in 1966, referencing the biblical figure Job, who was afflicted with "loathsome sores" from head to toe, mirroring the severe recurrent skin abscesses often seen in patients. Another historical name, Buckley Syndrome, honors Dr. Rebecca Buckley, who provided the first comprehensive clinical description of the condition in 1972. You may also encounter the abbreviation HIES in medical records or research papers.
Classification and Nomenclature
The evolution of the name Hyper IgE Syndrome reflects our growing understanding of its genetic origins. Historically, these conditions were grouped together based solely on elevated serum IgE levels and skin/lung involvement. Today, the medical community distinguishes between subtypes based on the underlying gene:
- AD-HIES: Caused by mutations in the STAT3 gene.
- AR-HIES: Caused by mutations in DOCK8 or PGM3 genes.
These classifications are reflected in the OMIM (Online Mendelian Inheritance in Man) database, where the syndrome is categorized by these specific genetic markers rather than just the clinical presentation of Hyper IgE Syndrome.
Why Multiple Names Exist
The variety of names stems from the historical practice of naming rare diseases after the researchers who first identified them or after clinical symptoms. As we moved from symptom-based diagnosis to molecular genetic testing, the nomenclature shifted to reflect the specific genetic etiology of Hyper IgE Syndrome. While "Job Syndrome" remains a widely recognized clinical term, Hyper IgE Syndrome is the preferred designation in current clinical practice because it accurately describes the immunological hallmark of the condition while allowing for genetic sub-classification.
Medical Disclaimer: This information is for educational purposes and should not replace professional medical advice, diagnosis, or treatment. Always consult with your immunologist or healthcare provider regarding specific symptoms or genetic test results.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Hyper-IgE Syndrome
- Orphanet: Autosomal dominant hyper-IgE syndrome
- Online Mendelian Inheritance in Man (OMIM): Hyper-IgE recurrent infection syndrome
