Job's syndrome

(Job, character in the Old Testament who suffered from skin disease and other misfortunes) (also called hyperimmunoglobulinaemia E syndrome), an immune deficiency disorder secondary to deficient T cell production of gamma interferon, a cytokine which enhances granulocytic chemotaxis and suppresses IgE production. Children with the syndrome have very high IgE serum levels. Clinically they get severe recurrent infection, mainly due to Candida and staphylococci, presenting as skin, sinus and pulmonary infection. Radiologically, the appearances are those of severe pneumonia, often complicated by abscess formation (Fig.1), sinus disease or osteomyelitis. See Buckleys syndrome

HC