Dermatologic Manifestations of Job Syndrome Workup

Updated: Mar 30, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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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.

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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. [46]

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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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