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Updated: Nov 6, 2025
06:53
Cell Population Analyses During Skin Carcinogenesis
Published on: August 21, 2013
12.7K
Basal cell nevus syndrome with excessive basal cell carcinomas
Choon Soo Kim1, Young Cheon Na1
1Department of Plastic and Reconstructive Surgery, Wonkwang University Hospital, Iksan, Korea.
Archives of Craniofacial Surgery
|May 7, 2021
Summary
Basal cell nevus syndrome (BCNS), a rare genetic disorder, can manifest with multiple basal cell carcinomas. This case highlights a patient diagnosed with Gorlin syndrome, successfully treated with surgical excision of facial lesions.
Area of Science:
- Genetics
- Dermatology
- Oncology
Background:
- Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder.
- It affects approximately 1 in 60,000 individuals, with varying prevalence reported globally.
Observation:
- A 40-year-old male presented with multiple facial pigmented macules, jaw cysts, bifid ribs, and falx cerebri calcification.
- These clinical features were consistent with the diagnostic criteria for Basal cell nevus syndrome.
Findings:
- Surgical excision and biopsy of facial lesions revealed basal cell carcinomas in most samples.
- Histopathological confirmation of basal cell carcinomas in the context of BCNS.
Implications:
- Early diagnosis and surgical management are crucial for patients with Basal cell nevus syndrome.
- This case underscores the importance of recognizing the multifaceted clinical presentation of Gorlin syndrome.
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