A Rare Case of Gorlin-Goltz Syndrome Presenting with Multiple Basal Cell Carcinomas in a Young Female

Abstract

Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare autosomal dominant disorder characterized by multiple basal cell carcinomas (BCCs), odontogenic keratocysts, skeletal anomalies, and developmental abnormalities. We report an 18-year-old female who presented with multiple hyperpigmented, well-defined nodules over the abdomen, lower back, and thighs since childhood. Histopathology of a nodular lesion confirmed adenoid BCC. Radiological imaging revealed odontogenic keratocysts and impacted teeth, establishing a diagnosis of Gorlin syndrome. This case underscores the importance of recognizing cutaneous and systemic features for early diagnosis, genetic counseling, and multidisciplinary management.So it is important to look for syndromic association if a patient present with cutaneous tumour in young age.

Keywords: Gorlin syndrome, nevoid basal cell carcinoma syndrome, basal cell carcinoma, odontogenic keratocyst.