Hyper IgE Disorder Associated with Multiple Fused Primary Teeth: A Rare Clinical Occurrence.
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.
Professor, Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India.
Aj Sai
AS
Sankar
Professor and Head, Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur Andhra Pradesh, India.
Mg Manoj
MM
Kumar
Senior Lecturer, Department of Oral Medicine and Radiology, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India.
Y
Y
Samata
Senior Lecturer, Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur Andhra Pradesh, India.
K Srikanth
KS
Reddy
Hyperimmunoglobulin; Pneumonia; Syndrome.