title: Hyper-IgE syndrome: a case report.
abstract: The hyper-IgE syndrome (HIES) is a rare disorder characterized by pruritic dermatitis, recurrent Staphylococcus skin abscesses and extremely elevated levels of IgE in serum. In this report, an eleven-year-old-boy with hyper-IgE syndrome is presented. He had a coarse facial appearance, pruritic dermatitis, recurrent skin abscesses, pulmonary infection, a reduced rate of resorption of the roots of primary teeth and an elevated serum IgE concentration. The colonization of Candida albicans, Kiebsiella pneumoniae, Escherichia coli and Staphylococcus aureus were found as; (1x10(2) CFU), (2.2x10(4) CFU), (2.2x10(4) CFU) and (2.6x10(3) CFU) per ml saliva, respectively. Also the pulp of a deciduous molar was investigated with light and transmission electron microscope (TEM). As conclusion, treatment for this condition is lifelong administration of therapeutic doses of a penicillinase-resistant penicillin, with the addition of other antibiotics or anti-fungal agents as required for specific infections.
authors.affiliation: University of Istanbul, Faculty of Dentistry, Department of Pedodontics, Turkey. elifsepet@hotmail.com
authors.forename: E
authors.initials: E
authors.lastname: Sepet
authors.forename: D
authors.initials: D
authors.lastname: Ozdemir
authors.forename: N
authors.initials: N
authors.lastname: Aksakalli
authors.forename: G
authors.initials: G
authors.lastname: Külekçig
mesh_terms: D002648:Child; D015169:Colony Count, Microbial; D003782:Dental Pulp / Q000473:pathology; D019066:Facies; D006801:Humans; D007589:Job Syndrome / Q000097:blood / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D012391:Root Resorption; D012463:Saliva / Q000382:microbiology; D014080:Tooth Exfoliation / Q000503:physiopathology*; D014092:Tooth Root / Q000503:physiopathology; D014094:Tooth, Deciduous / Q000503:physiopathology*