job’s syndrome

Job’s syndrome or hyper-immunoglobulin E (IgE) syndrome (HIES) is an extremely rare primary immunodeficiency disease with an approximate annual incidence of less than 1/1000000. It is characterized by recurrent cold staphylococcal infections, unusual eczematous dermatitis, severe lung infections, and extensively high concentrations of the serum antibody IgE.

A typical case of Job’s syndrome with a family history of Kawasaki disease is presented in this study aiming at identifying the clinical features, investigational procedures, and management strategy, as well as evaluating the role of the ear, nose, and throat specialist and highlighting the probable relation between Job’s syndrome and Kawasaki disease.

In general, early detection with proper care can prevent the progression of Job syndrome. In addition, the initiated treatment at the first signs of infection is mandatory for preventing long-term complications. There is a probable relation between Job and Kawasaki which requires more consideration.