eMedicine Specialties > Dermatology > Allergy & Immunology

Job Syndrome: Differential Diagnoses & Workup

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Mordechai M Tarlow, MD, Physician, Department of Medicine, Section of Dermatology, Kimball Medical Center
Contributor Information and Disclosures

Updated: Jul 17, 2009

Differential Diagnoses

Atopic Dermatitis
Eosinophilic Pustular Folliculitis
Folliculitis
Gram-Negative Folliculitis
Onychomycosis

Workup

Laboratory Studies

  • By definition, hyper-IgE syndrome (HIE syndrome, Job syndrome) is characterized by an elevated serum IgE level.
    • Levels vary, but the vast majority of patients have indices greater than 2000 IU/mL, and many patients have levels as high as 50,000 IU/mL. (Normal values of serum IgE tend to be less than 10 IU/mL in an arithmetic distribution or less than 100 IU/mL after logarithmic conversion, although these values may vary among laboratories.)
    • Serum IgE values tend to fluctuate to some degree (most often by <50%), and, in some patients, disease activity can significantly decrease over the years.
    • A normal IgE level should not exclude Job syndrome in an adult.
  • Serum eosinophil counts are more than 2 standard deviations above the normal range of values in more than 90% of patients.
  • Elevated eosinophil counts can be found in secretion samples, including those obtained with abscess drainage and sputum samples in cases of bronchitis or pneumonia.
  • No correlation is observed between the level of serum IgE and the level of serum eosinophils, and fluctuations in these levels are not associated with infections or flares of the dermatitis.

Imaging Studies

  • Pulmonary imaging (eg, x-ray, CT) features typically reveal recurrent alveolar lung infections; pneumatoceles; and, rarely, pneumothorax.
  • Radiographs of the teeth indicate the dental development age.

Other Tests

  • Neutrophil chemotaxis may be assessed by means of in vitro examination of their ability to move toward a chemoattractant. Such chemoattractants include endotoxin-activated serum, sodium caseinate, and formylmethionine-leucine-phenylalanine (fMet-Leu-Phe).
  • Although results with these tests are most often abnormal when compared with control values, chemotactic responsiveness varies, and the magnitude of the defect is less than that in other disorders (eg, Chediak-Higashi syndrome).

Histologic Findings

Histologic examination of vesicopapules may reveal an eosinophil-rich infiltration around the hair follicles, similar to that of eosinophilic pustular folliculitis.

More on Job Syndrome

Overview: Job Syndrome
Differential Diagnoses & Workup: Job Syndrome
Treatment & Medication: Job Syndrome
Follow-up: Job Syndrome
References
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References

  1. Grimbacher B, Holland SM, Puck JM. Hyper-IgE syndromes. Immunol Rev. Feb 2005;203:244-50. [Medline].

  2. Minegishi Y, Karasuyama H. Genetic origins of hyper-IgE syndrome. Curr Allergy Asthma Rep. Sep 2008;8(5):386-91. [Medline].

  3. Ma CS, Chew GY, Simpson N, et al. Deficiency of Th17 cells in hyper IgE syndrome due to mutations in STAT3. J Exp Med. Jul 7 2008;205(7):1551-7. [Medline].

  4. Stiehm ER. Cytokine dysregulation in the hyperimmunoglobulinemia E syndrome. J Pediatr. Feb 2000;136(2):141-3. [Medline].

  5. Paslin D, Norman ME. Atopic dermatitis and impaired neutrophil chemotaxis in Job's syndrome. Arch Dermatol. Jun 1977;113(6):801-5. [Medline].

  6. Simon HU, Seger R. Hyper-IgE syndrome associated with an IL-4-producing gamma/delta(+) T-cell clone. J Allergy Clin Immunol. Jan 2007;119(1):246-8. [Medline].

  7. Vargas L, Patino PJ, Rodriguez MF, et al. Increase in granulocyte-macrophage-colony-stimulating factor secretion and the respiratory burst with decreased L-selectin expression in hyper-IgE syndrome patients. Ann Allergy Asthma Immunol. Sep 1999;83(3):245-51. [Medline].

  8. Shirafuji Y, Matsuura H, Sato A, Kanzaki H, Katayama H, Arata J. Hyperimmunoglobin E syndrome: a sign of TH1/TH2 imbalance?. Eur J Dermatol. Mar 1999;9(2):129-31. [Medline].

  9. Tanaka T, Takada H, Nomura A, Ohga S, Shibata R, Hara T. Distinct gene expression patterns of peripheral blood cells in hyper-IgE syndrome. Clin Exp Immunol. Jun 2005;140(3):524-31. [Medline].

  10. Hawn TR, Ozinsky A, Williams LM, et al. Hyper-IgE syndrome is not associated with defects in several candidate toll-like receptor pathway genes. Hum Immunol. Jul 2005;66(7):842-7. [Medline].

  11. Vigliante CE, Costello BJ, Quinn PD. Life-threatening cervicofacial infection in a child with hyperimmunoglobulin-E syndrome. J Oral Maxillofac Surg. May 2001;59(5):561-5. [Medline].

  12. O'Connell AC, Puck JM, Grimbacher B, et al. Delayed eruption of permanent teeth in hyperimmunoglobulinemia E recurrent infection syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Feb 2000;89(2):177-85. [Medline].

  13. Onal IK, Kurt M, Altundag K, Aksoy S, Dincer M, Gullu I. Peripheral T-cell lymphoma and Job's syndrome: a rare association. Med Oncol. 2006;23(1):141-4. [Medline].

  14. Ling JC, Freeman AF, Gharib AM, et al. Coronary artery aneurysms in patients with hyper IgE recurrent infection syndrome. Clin Immunol. Mar 2007;122(3):255-8. [Medline].

  15. Sarmento KM Jr, Tomita S, Caliman e Gurgel JD. Association between nasal polyposis, Dubowitz syndrome and hyper-IgE syndrome. Int J Pediatr Otorhinolaryngol. May 2008;72(5):711-4. [Medline].

  16. Borges WG, Hensley T, Carey JC, Petrak BA, Hill HR. The face of Job. J Pediatr. Aug 1998;133(2):303-5. [Medline].

  17. Freeman AF, Domingo DL, Holland SM. Hyper IgE (Job's) syndrome: a primary immune deficiency with oral manifestations. Oral Dis. Jan 2009;15(1):2-7. [Medline].

  18. Grimbacher B, Holland SM, Gallin JI, et al. Hyper-IgE syndrome with recurrent infections--an autosomal dominant multisystem disorder. N Engl J Med. Mar 4 1999;340(9):692-702. [Medline].

  19. Grimbacher B, Schaffer AA, Holland SM, et al. Genetic linkage of hyper-IgE syndrome to chromosome 4. Am J Hum Genet. Sep 1999;65(3):735-44. [Medline].

  20. Tanaka H, Ito R, Onodera N, Waga S. Efficacy of long-term sulfamethoxazole-trimethoprim therapy in a boy with hyperimmunoglobulin E syndrome. Tohoku J Exp Med. Sep 1998;186(1):61-6. [Medline].

  21. Kojima K, Inoue Y, Katayama Y, et al. Improvement with disodium cromoglycate of neutrophil phagocytosis and respiratory burst activity in a patient with hyperimmunoglobulin E syndrome. Allergy. Nov 1998;53(11):1101-3. [Medline].

  22. Wakim M, Alazard M, Yajima A, Speights D, Saxon A, Stiehm ER. High dose intravenous immunoglobulin in atopic dermatitis and hyper-IgE syndrome. Ann Allergy Asthma Immunol. Aug 1998;81(2):153-8. [Medline].

  23. Hori J, Yamaguchi S, Watanabe M, Osanai H, Hori M. Fournier gangrene associated with hyper IgE syndrome (Job syndrome). Int J Urol. Apr 2008;15(4):372-3. [Medline].

  24. Donabedian H, Gallin JI. The hyperimmunoglobulin E recurrent-infection (Job's) syndrome. A review of the NIH experience and the literature. Medicine (Baltimore). Jul 1983;62(4):195-208. [Medline].

  25. Erlewyn-Lajeunesse MD. Hyperimmunoglobulin-E syndrome with recurrent infection: a review of current opinion and treatment. Pediatr Allergy Immunol. Aug 2000;11(3):133-41. [Medline].

  26. Van Eendenburg JP, Smitt JH, Weening RS. Hyperimmunoglobulin E recurrent infection (Job's) syndrome. Br J Dermatol. Oct 1991;125(4):397. [Medline].

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Further Reading

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Keywords

Job syndrome, hyperimmunoglobulin E syndrome, hyper IgE syndrome, hyper-IgE syndrome, HIE syndrome, Job's syndrome, IgE, immunoglobulin E

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Mordechai M Tarlow, MD, Physician, Department of Medicine, Section of Dermatology, Kimball Medical Center
Mordechai M Tarlow, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for MOHS Surgery, American Society of Cosmetic Dermatology and Aesthetic Surgery, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Bernice R Krafchik, MBChB, FRCPC, Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto
Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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