International Journal of Clinical Pediatric Dentistry

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VOLUME 3 , ISSUE 3 ( September-December, 2010 ) > List of Articles

CASE REPORT

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

Yalamanchili Samatha, MG Manoj Kumar, Y Samata, K Srikanth Reddy

Citation Information : Samatha Y, Manoj Kumar M, Samata Y, Srikanth Reddy K. Hyper IgE Disorder Associated with Multiple Fused Primary Teeth: A Rare Clinical Occurrence. Int J Clin Pediatr Dent 2010; 3 (3):215-218.

DOI: 10.5005/jp-journals-10005-1081i

License: CC BY-NC 4.0

Published Online: 00-00-0000


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.


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