Extensive Flexural Basal Cell Carcinomas Revealing Gorlin-Goltz Syndrome With Marked Histopathologic Diversity: A...

Gorlin-Goltz syndrome is a rare autosomal dominant disorder characterized by multiple basal cell carcinomas (BCCs), odontogenic keratocysts, and skeletal anomalies. BCCs in this syndrome typically involve photo-exposed areas, whereas predominant flexural and intertriginous involvement remains exceptionally rare. We report the case of a 42-year-old Moroccan man with Fitzpatrick phototype IV who presented with a more than 30-year history of progressively increasing pigmented and erythematous lesions. Clinical examination revealed more than 100 papules, plaques, and nodules involving the face, axillary folds, inguinal folds, and umbilical region. Some inguinal lesions became ulcerated and symptomatic, with pain and bleeding. Dermoscopy showed characteristic features of BCC, including arborizing vessels, blue-gray ovoid nests, and globules, leading to multiple biopsies that confirmed the diagnosis. Histopathological examination identified three distinct subtypes, nodular, adenoid-cystic, and fibroepithelioma of Pinkus, highlighting marked histopathological heterogeneity. A panoramic jaw radiograph demonstrated multiple odontogenic keratocysts, while chest radiography revealed bifid ribs, fulfilling major diagnostic criteria for Gorlin-Goltz syndrome. Genetic testing was not performed because of limited availability. The patient underwent surgical excision of the most symptomatic lesions. Targeted therapy with the Hedgehog pathway inhibitor vismodegib was considered but was not accessible in our setting. This case highlights a rare flexural-predominant phenotype of Gorlin-Goltz syndrome and emphasizes the importance of recognizing atypical non-sun-exposed presentations to avoid diagnostic delay and ensure appropriate multidisciplinary management.