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Dermatologic Manifestations of Job Outcomes

HIE syndrome has variable expressivity and is associated with several abnormalities. The most common findings are recurrent skin abscesses (hence, the title Job syndrome), pneumonia with pneumatocele growth, and higher serum levels of IgE. Facial, dental, and skeletal features are also related to this syndrome. Autosomal recessive patients generally have severe molluscum contagiosum and other viral infections and may develop severe neurological complications. These patients also lack dental or skeletal involvement and don’t develop lung cysts. Some authorities believe 2 individual syndromes exist, none.

Type 1 HIE syndrome shows abnormalities in a number of systems, including the skeletal, dental, and immune systems, whereas type 2 HIE syndrome reveals abnormalities restricted to the immune system. Cytokine responses in both kinds of HIE syndrome demonstrated severe defects resulting in diminished T-helper type 17 function. Another study credited lack of Th17 cells in HIE syndrome to mutations in STAT3 in the vast majority of evaluated patients. But, defective Th17 responses could be viewed in classic illness without STAT3 mutations, together with the degree of the faulty Th17 response postulated to find out the clinical phenotype.

Pathophysiology

The pathophysiology of Job syndrome (HIE syndrome, or hyper-IgE syndrome) isn’t completely understood. Patients always have a bad, delayed hypersensitivity reaction to antigens. This delayed response is related to alterations in T-lymphocyte inhabitants and various interleukin and cytokine abnormalities. Among the first reports on the pathophysiology of Job syndrome described a chemotactic defect in neutrophils. The bad production of interferon-gamma in reaction to IL-12 leads to the marked elevation of IgE levels (by way of unopposed IL-4 activity).

Other aspects in the abnormal immunologic response are described. Patients with HIE syndrome have elevated levels of granulocyte-macrophage colony-stimulating variable, which might also explain the decreased chemotaxis and increased oxygen radical production and tissue damage. Deficient suppressor T-cell amounts and action and an imbalance in helper T cell type 1 (TH1) and helper T cell type 2 (TH2) also may play a part in an abnormal reaction.