Hyperimmunoglobulin E syndrome
autosomal dominant form called Job's syndrome or Buckley syndrome, is a heterogeneous group of immune disorders
Hyperimmunoglobulinemia E syndrome (HIES), of which the autosomal dominant form is called Job's syndrome or Buckley syndrome, is a heterogeneous group of immune disorders. Job's is also very rare at about 300 cases currently in literature.
Presentation
It is characterized by recurrent "cold" staphylococcal infections (due to impaired recruitment of neutrophils), unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles (balloon-like lesions that may be filled with air or pus or scar tissue) and very high (> 2000 IU/mL or 4800 mcg/L) concentrations of the serum antibody IgE. Inheritance can be autosomal dominant or autosomal recessive. Many patients with autosomal dominant STAT3 hyper-IgE syndrome have characteristic facial and dental abnormalities, fail to lose their primary teeth, and have two sets of teeth simultaneously.
Pathophysiology
Abnormal neutrophil chemotaxis due to decreased production of interferon gamma by T lymphocytes is thought to cause the disease.
Both autosomal dominant and recessive inheritance have been described:
Autosomal dominant:
STAT3 may present as HIES with characteristic facial, dental, and skeletal abnormalities that has been called Job's Syndrome. A common mnemonic used to remember the symptoms is FATED: coarse or leonine facies, cold staph abscesses, retained primary teeth, increased IgE, and dermatologic problems [eczema]. The disease was linked to mutations in the STAT3 gene after cytokine profiles indicated alterations in the STAT3 pathway. This altered pathway directly reduces the modulation capacity of interleukins 6 and 10 which, respectively, inhibit the genesis of Th17 cells that, in tandem with CD4 cells, protect against bacterial and fungal infections, and foster the inappropriate immune responses exhibited by those with Job Syndrome.
Autosomal recessive:
DOCK8 - DOCK8 Immunodeficiency Syndrome (DIDS) presents primarily with immune effects including HEIS. Eczema is prominent, food and environmental allergies are common, and asthma and anaphylaxis has been variably reported.
The public source identifies “Hyperimmunoglobulin E syndrome” as autosomal dominant form called Job's syndrome or Buckley syndrome, is a heterogeneous group of immune disorders. This brief keeps that definition visible, then builds a research path around Hyperimmunoglobulin, syndrome and autosomal.
Why this record matters
A short description can identify a subject without explaining its stakes. For “Hyperimmunoglobulin E syndrome”, the useful work is to connect “autosomal dominant form called Job's syndrome or Buckley syndrome, is a heterogeneous group of immune disorders” to the records capable of establishing context and consequence.
Vocabulary and entity names are the principal evidence signals here, because they determine the precision of every later search. The source revision retrieved here is dated Jun 9, 2026. The linked authority identifier is Q2336873. None of the 0 selected statements returned an explicit reference. The first chronological checks are 2000.
A concise general-reference account can conceal disagreements about scope, terminology or the weight assigned to individual sources. The source lead contains qualifying language; that uncertainty should survive quotation, summary and reuse. Authority statements aid reconciliation but still require their own references, qualifiers and ranks to be checked.
How to read it
Use the entry as an orientation point, then follow its citations and revision history. Names, dates and institutional relationships should be checked against the original record.
- Subject orientation
- Search vocabulary
- Locating named sources
The closest primary source, responsible institution and strongest cited specialist reference.
Three-step research path
- Establish the record: confirm the title “Hyperimmunoglobulin E syndrome”, its source revision and the description used here.
- Expand the search: follow Hyperimmunoglobulin E syndrome primary sources, Hyperimmunoglobulin E syndrome archive and Hyperimmunoglobulin research across catalogues and specialist indexes.
- Test the account: compare the strongest cited source with the responsible institution’s current record and note any disagreement.
Questions for further research
- Which source most directly establishes the central claim about “Hyperimmunoglobulin E syndrome”?
- What terminology or title could unlock a more precise catalogue search?
- Which institution is responsible for the underlying evidence?
Search terms from this dossier
This entry incorporates text from “Hyperimmunoglobulin E syndrome” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.