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Basal cell nevus syndrome. A case report
International Journal of Oral Surgery
|February 1, 1979
Summary
Basal cell nevus syndrome, a genetic disorder, can manifest with multiple dentigerous cysts in the jawbones. This case highlights the syndrome
Area of Science:
- Dentistry
- Genetics
- Dermatology
Background:
- Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder.
- It is characterized by a wide spectrum of developmental abnormalities, including various tumors and cysts.
Observation:
- An 11-year-old boy presented with multiple dentigerous cysts in the maxilla and mandible.
- Additional clinical findings were noted in the facial structure, plantar skin, and skeletal system.
Findings:
- The constellation of clinical features strongly suggested basal cell nevus syndrome.
- Genetic confirmation was supported by the presence of similar abnormalities in the patient's father and brother.
Implications:
- Early diagnosis of BCNS is crucial for monitoring and managing associated risks, such as basal cell carcinomas and medulloblastomas.
- This case underscores the importance of recognizing the diverse clinical manifestations of BCNS for timely intervention and genetic counseling.