Encyclopedia of Medical Immunology

2014 Edition
| Editors: Ian R. Mackay, Noel R. Rose, Dennis K. Ledford, Richard F. Lockey

Hyper-IgE Syndrome

  • Thomas Eiwegger
  • Zsolt Szépfalusi
Reference work entry
DOI: https://doi.org/10.1007/978-1-4614-9194-1_80


Hyper-IgE Syndrome (HIES); Job´s Syndrome


Hyper-IgE syndrome is an inherited multisystem disease characterized by the classical triad of abscesses, pneumonia, and extremely elevated serum IgE.

Immunological and Non-Immunological Clinical Features

Job´s syndrome was first reported 1966 by Davis et al. on the basis of the case reports of two young girls with eczema, cold abscesses of the skin, and pneumonias (Davis et al. 1966). It was named Job´s syndrome in reference to the biblical Job whose body was affected with boils. Based on inheritance, an autosomal dominant (AD-HIES) and an autosomal recessive (AR-HIES) form of hyperimmunoglobulin E syndrome have been reported.


The autosomal dominant form of hyper-IgE syndrome is a multisystem disorder. In addition to the classical triad (cold abscesses, pneumonia with pneumatocele, and extremely elevated IgE), it displays numerous immunological and non-immunological characteristics (Table 1). Among the...
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  1. Barlogis V, Galambrun C, et al. Successful allogeneic hematopcbdoietic stem cell transplantation for DOCK8 deficiency. J Allergy Clin Immunol. 2011;128(2):420–2 e422.PubMedCrossRefGoogle Scholar
  2. Bilora F, Petrobelli F, et al. Moderate-dose intravenous immunoglobulin treatment of Job’s syndrome. Case report. Minerva Med. 2000;91(5–6):113–6.PubMedGoogle Scholar
  3. Bittner TC, Pannicke U, et al. Successful long-term correction of autosomal recessive hyper-IgE syndrome due to DOCK8 deficiency by hematopoietic stem cell transplantation. Klin Padiatr. 2010;222(6):351–5.PubMedCrossRefGoogle Scholar
  4. Davis SD, Schaller J, et al. Job’s syndrome. Recurrent, “cold”, staphylococcal abscesses. Lancet. 1966;1(7445):1013–5.PubMedCrossRefGoogle Scholar
  5. Engelhardt KR, McGhee S, et al. Large deletions and point mutations involving the dedicator of cytokinesis 8 (DOCK8) in the autosomal-recessive form of hyper-IgE syndrome. J Allergy Clin Immunol. 2009;124(6):1289–302 e1284.PubMedCrossRefPubMedCentralGoogle Scholar
  6. Gatz SA, Benninghoff U, et al. Curative treatment of autosomal-recessive hyper-IgE syndrome by hematopoietic cell transplantation. Bone Marrow Transpl. 2011;46(4):552–6.CrossRefGoogle Scholar
  7. Holland SM, DeLeo FR, et al. STAT3 mutations in the hyper-IgE syndrome. N Engl J Med. 2007;357(16):1608–19.PubMedCrossRefGoogle Scholar
  8. Levy DE, Loomis CA. STAT3 signaling and the hyper-IgE syndrome. N Engl J Med. 2007;357(16):1655–8.PubMedCrossRefGoogle Scholar
  9. McDonald DR, Massaad MJ, et al. Successful engraftment of donor marrow after allogeneic hematopoietic cell transplantation in autosomal-recessive hyper-IgE syndrome caused by dedicator of cytokinesis 8 deficiency. J Allergy Clin Immunol. 2010;126(6):1304–5.PubMedCrossRefPubMedCentralGoogle Scholar
  10. Metin A, Tavil B, et al. Successful bone marrow transplantation for DOCK8 deficient hyper IgE syndrome. Pediatr Transpl. 2012;16(4):398–9.CrossRefGoogle Scholar
  11. Minegishi Y, Saito M, et al. Dominant-negative mutations in the DNA-binding domain of STAT3 cause hyper-IgE syndrome. Nature. 2007;448(7157):1058–62.PubMedCrossRefGoogle Scholar
  12. Puel A, Cypowyj S, et al. Chronic mucocutaneous candidiasis in humans with inborn errors of interleukin-17 immunity. Science. 2011;332(6025):65–8.PubMedCrossRefPubMedCentralGoogle Scholar
  13. Renner ED, Torgerson TR, et al. STAT3 mutation in the original patient with Job’s syndrome. N Engl J Med. 2007;357(16):1667–8.PubMedCrossRefGoogle Scholar
  14. Scarabelli TM, Townsend PA, et al. Amino acid supplementation differentially modulates STAT1 and STAT3 activation in the myocardium exposed to ischemia/reperfusion injury. Am J Cardiol. 2008;101(11A):63E–8.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of Pediatrics and Adolescent MedicineMedical University of ViennaViennaAustria
  2. 2.Division of Pediatric Pulmonology, Allergy and Endocrinology, Department of Pediatrics and Juvenile MedicineMedical University of ViennaViennaAustria