Abstract:

  Backround: Basal cell nevus syndrome (BCNS) is an autosomal dominant inherited condition.

  The syndrome presents with multiple jaw cysts (more often,OKC), skeletal abnormalities ,ectopic intracranial calcification , multiple basal cell carcinomas of the skin . The prevalence of Gorlin syndrome is estimated to be about 1/60000 to 1/120000 . This syndrome if often distinguished in third decade of life.In this article a rare case of Basal cell nevus syndrome is reported.

  Case presentation : The case was a 23 year- old male patient with the chief complaint of exiting pus from the left side of his mandible ( Buccal of first molar) who was referred to Islamic Azad University (Dental Branch) Radiology Dept. In radiographic assessment (panoramic radiography) multiple cystic lesions and thus impacted teeth in maxilla were seen . In histopathological view, epithelium of odontogenic cyst , keratin debries and Palisaded basal cells were seen in both jaws. The definitive diagnosis was multiple odontogenic keratocysts. Treatment was done first by marsupialization , enuclation and curettage. In three months follow up, recurrance wasnot seen.

  Conclusion: Early diagnosis of Basal Cell Nevus Syndrome (BCNS) by dentists or dental specialists play an important role in patients' prognosis.