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Basal cell nevus syndrome: a case report
T J Ocholla1, S W Guthua, S S Kimaro
1Division of Oral and Maxillofacial Surgery, University of Nairobi, College of Health Sciences, Kenya.
East African Medical Journal
|November 1, 1994
Summary
A rare genetic disorder, basal cell nevus syndrome, was diagnosed in a 13-year-old Kenyan girl presenting with multiple cysts and skin lesions. This marks the first reported case in Kenya, highlighting the need for vigilant diagnosis.
Area of Science:
- Medical Genetics
- Dermatology
- Odontology
Background:
- Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder.
- It is characterized by a spectrum of developmental abnormalities, including multiple basal cell carcinomas, jaw cysts, and skeletal anomalies.
Observation:
- A 13-year-old Kenyan girl presented with multiple mandibular and maxillary cysts, characteristic cutaneous lesions, and mandibular prognathism.
- Clinical and radiographic examinations were crucial in identifying the characteristic features of the syndrome.
Findings:
- The patient was diagnosed with basal cell nevus syndrome based on the presented clinical and radiographic features.
- This case represents the first documented instance of basal cell nevus syndrome in Kenya.
Implications:
- This case underscores the importance of comprehensive clinical evaluation and radiographic screening in diagnosing rare genetic syndromes.
- Early diagnosis and management of basal cell nevus syndrome can potentially improve patient outcomes and prevent complications.