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Hyper IgE Disorder Associated with Multiple Fused Primary Teeth: A Rare Clinical Occurrence.
Aj Sai Sankar1, Mg Manoj Kumar2, Y Samata3
1Professor, Department of Pedodontics and Preventive Dentistry, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India.
International Journal of Clinical Pediatric Dentistry
|September 13, 2016
Summary
Hyperimmunoglobulin E syndrome (HIES) can present with rare dental anomalies like fused primary teeth. Early recognition of these oral manifestations is crucial for managing this multisystem disorder.
Area of Science:
- Immunology
- Genetics
- Dentistry
Background:
- Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency affecting multiple systems, including immune, skeletal, and connective tissues.
- Oral manifestations of HIES are not well-documented, necessitating further research into associated dental findings.
Observation:
- A 6-year-old boy with suspected autosomal recessive HIES presented with recurrent pneumonia and skin infections.
- The patient exhibited multiple fused primary teeth, a dental anomaly rarely associated with HIES, also known as JOB syndrome.
Findings:
- The case highlights a rare association between autosomal recessive HIES and fused primary teeth.
- This observation expands the known spectrum of clinical manifestations in HIES, particularly concerning dental development.
Implications:
- Early identification of oral symptoms in HIES can aid in timely diagnosis and intervention.
- Prompt management, including long-term antibiotic prophylaxis, is essential to reduce morbidity in patients with HIES.
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