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 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.

Le syndrome de Job-Buckley : à propos d’un cas et revue de la littérature

“…Des infections candidosiques chroniques, essentiellement liées à Candida albicans, tant au niveau cutané qu'au niveau oropharyngé sont également fréquemment rapportées dans ce syndrome [15,16].…”

Hyperimmunoglobulin E syndrome: Two cases and a review of the literature

“…Reported to be safe and effective, life-long penicillinase-resistant penicillins with intermittent culture-directed antibiotics and antifungals as required have been used in HIES patients. 59 Another growing problem that complicates treatment is the development of MRSA. MRSA is classically seen as a nosocomial infection in hospitals, but is becoming a more common occurrence in the community setting.…”

Hyper IgE Disorder Associated with Multiple Fused Primary Teeth: A Rare Clinical Occurrence

“…1 It is characterized by a triad of eczema, recurrent skin and lung infections, and a high serum level of IgE. The most consistent laboratory abnormalities in HIES are high serum IgE and eosinophilia.…”

“…Low or absent salivary and plasma anti-staphylococcal IgA likely contributes to the propensity to mucosal infections. 1 …”

“…A coarse facial appearance, hyper extensibility of the joints, bone fractures, craniosytosis and prominent triangular mandible have been reported in many cases of HIES. 1 The name Job’s syndrome was given by Davis in 1966 and Buckely subsequently related the condition to elevated levels of IgE. 2 …”