Job syndrome

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Job syndrome2016-12-25T07:48:53+00:00

Job syndrome 

Job syndrome image from New Medical Terms

Job

IMMUNOLOGY

An immunodeficiency disorder*, characterised by extremely high serum levels of immunoglobulin E, a leukocyte chemotaxis defect, and recurrent staphylococcal infections.

*The inheritance pattern of hyperimmunoglobulin E syndrome (Job syndrome) is either autosomal dominant (OMIM:147060) and due to a defect of STAT3, or autosomal recessive (OMIM:243700), due to defect of either DOCK8 or, rarely, a defect of TYK2

Clinical findings Recurrent otitis media, sinusitis, severe staphylococcal infections, chronic eczema and recurring abscesses of lungs, skin, joints.

Pathogenesis HIES/Job syndrome is a disorder of neutrophil oxidative metabolism

Note: The biblical Job was cursed and covered with boils–abscesses from head to toe–one of the calamities he suffered to test his faith in God. Hyperimmunoglobulin E syndrome is the accepted synonym for the biblical Job syndrome, because of the characteristic multiple recurring abscesses. However, because the description of his affliction was vague, medical historians have variously attributed the syndrome to syphilis, yaws, leprosy, smallpox, pemphigus, dermatitis herpetiformis, pellagra, and scurvy. The first formally described Job syndrome (The Lancet 1966; 1:1013) occurred in red-headed girls, it also occurs in males and in other hair colours. 

Synonyms Buckley syndrome, hyperimmunoglobulin E syndrome, Job’s disease

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