Job’s Syndrome With a Family History of Kawasaki Disease: A Case Presentation and Review of Literature

Background: Job’s syndrome or hyper-immunoglobulin E (IgE) syndrome (HIES) is an extremely rareprimary immunodeficiency disease with an approximate annual incidence of less than ۱/۱ ۰۰۰ ۰۰۰.It is characterized by recurrent cold staphylococcal infections, unusual eczematous dermatitis,severe lung infections, and extensively high concentrations of the serum antibody IgE.Case Presentation: A typical case of Job’s syndrome with a family history of Kawasaki disease ispresented in this study aiming at identifying the clinical features, investigational procedures, andmanagement strategy, as well as evaluating the role of the ear, nose, and throat specialist andhighlighting the probable relation between Job’s syndrome and Kawasaki disease.Conclusions: In general, early detection with proper care can prevent the progression of Jobsyndrome. In addition, the initiated treatment at the first signs of infection is mandatory forpreventing long-term complications. There is a probable relation between Job and Kawasaki whichrequires more consideration.

کلیدواژه ها:

Job’s syndrome ، Kawasaki ، Neck mass ، Hyper-immunoglobulin E syndrome ، Primary immunodeficiency