Hyper lgE syndrome (HIE): Report of 11 cases

Abstract

HIE is a primary immunodeficiency characterized by recurrent staphylococcal abscesses and markedly elevated serum IgE concentrations. These abcesses involve the skin, lungs, joints, and other sites, and there is a unique tendency to form pneumatoceles following staphylococcal pneumonias. Although, there is as yet no clearly defined immunologic cause for this rare syndrome, but some form of T-Cell defect may be cause, leading to matkedly elevation of IgE and eosinophillia. In this study clinical and immunopathological aspects of HIE syndrome in 11 patients have been evaluated