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 elevenyear-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; (1x102 CFU), (2.2x104 CFU), (2.2x104 CFU) and (2.6x103 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.

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