(Last Updated On: December 14, 2018)
Authors: Kariza Valerie L. De Vera, MD, Rogelio A. Balagat, MD, FPCP, FPDS, FPRA

Abstract

Introduction: Basal cell nevus syndrome is a rare autosomal dominant disorder with a prevalence ranging from 1 in 60,000 to 1 in 120,000. It is associated with a panoply of abnormalities including developmental anomalies and postnatal tumors such as basal cell carcinoma.

Case summary: We report a 50-year old Filipina who suffered from multiple recurrent pigmented papules and plaques on the face, first appearing at age 20. Some lesions enlarged into tumors and spontaneously bled and ulcerate. She underwent 2 excisions 21 years apart, each time showing the same histopathological report of basal cell carcinoma. Her mother had basal cell carcinoma. Both her hands showed palmar pits. She had a bifid left 3rd rib on chest x-ray. A skin biopsy also showed basal cell carcinoma.

Conclusion: This case illustrates how a case of basal cell nevus syndrome was diagnosed: basal cell carcinoma at a young age, a first-degree relative with the same tumor, palmar pits, and a bifid rib. Suspect basal cell nevus syndrome in a patient with multiple basal cell carcinoma arising at an early age.

 

Citation

 

Keywords

Basal Cell Nevus Syndrome; Basal Cell Carcinoma; Gorlin Syndrome

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