BACKGROUND: Gorlin-Goltz syndrome (GGS) is an autosomal dominant disorder mainly characterized by the presence of multiple basal cell carcinomas (BCC), odontogenic keratocysts of the jaw, and volar pits. This syndrome is associated with a wide spectrum of developmental anomalies and neoplasms. OBJECTIVE: To describe dermoscopic characteristics of cutaneous GGS lesions performing histopathologic correlation. MATERIALS AND METHODS: Cutaneous lesions and tumors from five patients affected by GGS were included. Clinical and dermoscopy images were obtained and excision with ulterior histopathology performed in suspicious tumors. RESULTS: With dermoscopy, BCCs can be detected in early stages by the presence of blue-gray globules in lesions less than 3 mm in diameter. In larger lesions, arborizing telangiectasia may also be present. Acral pits that are often overlooked during physical examination have a characteristic dermoscopy with red globules that are mainly distributed in parallel lines inside flesh-colored, irregular-shaped, and slightly depressed lesions. Dermoscopy improves the visualization of these pits. CONCLUSION: Dermoscopy can help in the diagnosis of the GGS as well as in the management of affected patients.
BACKGROUND: Gorlin-Goltz syndrome (GGS) is an autosomal dominant disorder mainly characterized by the presence of multiple basal cell carcinomas (BCC), odontogenic keratocysts of the jaw, and volar pits. This syndrome is associated with a wide spectrum of developmental anomalies and neoplasms. OBJECTIVE: To describe dermoscopic characteristics of cutaneous GGS lesions performing histopathologic correlation. MATERIALS AND METHODS: Cutaneous lesions and tumors from five patients affected by GGS were included. Clinical and dermoscopy images were obtained and excision with ulterior histopathology performed in suspicious tumors. RESULTS: With dermoscopy, BCCs can be detected in early stages by the presence of blue-gray globules in lesions less than 3 mm in diameter. In larger lesions, arborizing telangiectasia may also be present. Acral pits that are often overlooked during physical examination have a characteristic dermoscopy with red globules that are mainly distributed in parallel lines inside flesh-colored, irregular-shaped, and slightly depressed lesions. Dermoscopy improves the visualization of these pits. CONCLUSION: Dermoscopy can help in the diagnosis of the GGS as well as in the management of affected patients.
Authors: Suha N Aloosi; Kawa A Mahmood; Shakhawan M Ali; Payman Kh Mahmud; Seerwan O Hasan; Hawbash O Muhamed Journal: Adv J Emerg Med Date: 2018-04-21