RAISED SERUM-IgE LEVELS AND DEFECTIVE NEUTROPHIL CHEMOTAXIS IN THREE CHILDREN WITH ECZEMA AND RECURRENT BACTERIAL INFECTIONS

Right-sided endocarditis in an adult patient with hyperimmunoglobulin-E syndrome successfully treated with high-dose daptomycin

“…HIES, which was first described in 1966 and was called Job's syndrome, is characterized by specific facial characteristics, together with the presence of increased IgE levels, eczema and recurrent infections, which are usually caused by Staphylococcus aureus [2][3][4][5] . The most characteristic facial feature is a broad nasal base and broad nasal bridge, protrusion of the forehead, wide outer canthal distances, and deep set eyes [6,7] .…”

“…Hyperimmunoglobulin-E syndrome (HIES) is a syndrome of recurrent staphylococcal abscesses, sinopulmonary infections, severe eczema and elevated levels of serum IgE [1,2] . Herein, we describe a patient with HIES and right-sided endocarditis due to Staphylococcus aureus.…”

Immunoglobulin E in primary immunodeficiency diseases

“…This raises the issue of whether excessive IgE levels are permissive for Staphylococcus aureus infections, or whether staphylococcal infections stimulate excessive production of IgE. The latter is contradicted by the fact that most patients (35). To avoid further confusion, the name ''hyperimmunoglobulin E recurrent infection syndrome'' (HIE or HIES) is now widely accepted.…”

“…The incidence of 20 clinical features associated with HIES was published, based on a cohort of 30 patients with clear-cut HIES (36). It became clear that the hyper-IgE recurrent infection syndrome (Online Mendelian Inheritance in Man (OMIM) database #147060, #243700) is a rare primary immunodeficiency characterized by recurrent staphylococcal skin abscesses, pneumonias with pneumatocele formation, extreme elevations of serum IgE, eosinophilia, and distinct abnormalities of the connective tissue, skeleton, and dentition (33)(34)(35)(36)(37)(38)(39). Most cases are sporadic.…”

“…Many studies have focused on the immune aspects of the disease, such as detection of eosinophilia in blood, sputum, and abscesses (51,69), defective granulocyte chemotaxis (34,35,40,70), abnormal T lymphocyte subsets (51,71), defective antibody production (72)(73)(74)(75), and decreased production of, or responsiveness to, cytokines such as IL-4 and interferon-gamma (IFN-c) (76)(77)(78)(79). Complement levels were normal when studied.…”