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Dermatologic Manifestations of Job Syndrome Workup

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 09, 2016
 

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.

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Imaging Studies

Pulmonary imaging (eg, radiography, CT scanning) features of Job syndrome (HIE syndrome, or hyper-IgE syndrome) typically reveal recurrent alveolar lung infections; pneumatoceles; and, rarely, pneumothorax.

Radiographs of the teeth indicate the dental development age.

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Other Tests

In Job syndrome (HIE syndrome, or hyper-IgE syndrome), 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).

A polymerase chain reaction (PCR)–based high-resolution DNA-melting assay scanning selected exons of the STAT3 gene may establish a rapid molecular diagnosis in many patients.[38]

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Histologic Findings

In Job syndrome (HIE syndrome, or hyper-IgE syndrome), histologic examination of vesicopapules may reveal an eosinophil-rich infiltration around the hair follicles, similar to that of eosinophilic pustular folliculitis.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Mordechai M Tarlow, MD Clinical Assistant Professor of Dermatology, University of Pennsylvania School of Medicine

Mordechai M Tarlow, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, American College of Aesthetic and Cosmetic Physicians; American Society of Aesthetic/Cosmetic Physicians, American Medical Association, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania 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, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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