Peutz-Jeghers syndrome in a 14-year-old boy: case report and review of the literature.
Peutz-Jeghers syndrome (PSJ) is a relatively rare but well-recognized condition, with a prevalence of approximately one in 120,000 births in the USA. It is generally inherited as an autosomal dominant trait, although 35% of cases are new mutations. This disorder is characterized by melanocytic macules on the hands, feet, peri-oral skin and oral mucosa, and multiple gastrointestinal hamartomatous polyps. People with PSJ have an increased risk for developing a variety of malignant tumours. The aim of the present study was to report one case of PSJ in a 14-year-old boy with mucocutaneous pigmentation associated with duodenal hamartomatous polyps.
Pereira
C M
CM
Department of Semiology, School of Dentistry of Piracicaba, State University of Campinas, Piracicaba/SP, Brazil.
Coletta
R D
RD
Jorge
J
J
Lopes
M A
MA
D000293:Adolescent; D006801:Humans; D007417:Intestinal Polyps / Q000209:etiology / Q000473:pathology; D008046:Lip / Q000473:pathology; D008297:Male; D009059:Mouth Diseases / Q000209:etiology* / Q000473:pathology; D009061:Mouth Mucosa / Q000473:pathology*; D010580:Peutz-Jeghers Syndrome / Q000150:complications*