Document Type : Original paper

Authors

• Elham Sadat Afraz ¹
• Hadis Tajalli ²

¹ Assistant professor, Oral Medicine Department, Semnan University of Medical Sciences, Semnan, Iran

² Dentist, Semnan University of Medical Sciences, Semnan, Iran

Abstract

Background and Aim: Gorlin-Goltz syndrome (GGS), also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare multisystemic syndrome that can affect various of tissues and organs in the human body. Patients with this syndrome are at risk of developing basal cell skin cancer during puberty or early adulthood. Diagnosis of this syndrome is based on major and minor criteria. Early diagnosis and treatment of this syndrome is important to reduce the severity of complications including cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction due to jaw cysts.
Method: A 27-year-old male with no significant medical history except abdominal herniation visited Shahid Beheshti Dental Clinic. Previous clinicians had failed to diagnose to come up with a solid diagnosis.
Results: On extraoral examination, ocular hypertelorism was noticed. Multiple cyst like lesions were seen in panoramic graphy.also several dark nevus could be found on different regions of his body which after examination were diagnosed as basal cell carcinoma. Surgical enucleation was performed to remove cysts.
Conclusion: This case suggests that rare diseases such as the presence of multiple BCCs require further research and a multidisciplinary approach, since a rare and life-threatening disease can be the cause. Early diagnosis of Gorlin syndrome is of paramount importance to allow an appropriate therapeutic approach recommended by a multidisciplinary team. Patients with multiple skin lesions should be evaluated regularly by a general practitioner or dermatologist.

Keywords

• Gorlin-Goltz syndrome
• Nevoid basal cell carcinoma syndrome
• Odontogenic keratocyst
• Case reports
• Rare diseases

Main Subjects

• Medicine