Abstract: Peutze-Jeghers syndrome (PJS) is an autosomal dominant genetic disease caused by a germline mutation in the STK11/LKB1 gene and associated with pigmentation in oral mucosa,perioral skin ect. and gastrointestinal hamartomatous polyps.Most patients′ mucocutaneous pigmentations emerged before gastrointestinal polyps.In recent years, the number of patients with pigmentation in oral mucous and perioral skin have gradually increased in the department of stomatology.With no subjective symptoms,they are not diagnosed as having Peutze-Jeghers syndrome until gastrointestinal examination.To enable stomatology medical workers better understand and diagnose Peutz-Jeghers syndrome,this paper will expound and summarize PJS in terms of etiology,tumor susceptibility,clinical manifestation,diagnosis,examination and treatment,and its prevention.

Key words: Peutz-Jeghers syndrome, LKB1/STK11, diagnosis, therapy