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

Abstract

The hyperimmunoglobulin E syndrome (HIES) is a multisystem disorder that affects the dentition, skeleton, connective tissues and immune system. Little is known about oral manifestations of the syndrome. The purpose of this report was to describe a 6-year-old boy with suspected autosomal recessive HIES syndrome who had multiple fused primary teeth, which is a rare association with JOB syndrome. The patient gave a history of pneumonia and skin infections. Recognition of such case at an early age is necessary to reduce morbidity. As conclusion, treatment for this condition is life long administration of therapeutic doses of penicillinase-resistant penicillin, with the addition of other antibiotics or antifungal agents as required for specific infections.