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Peutz–Jeghers syndrome in a 14‐year‐old boy: case report and review of the literature
Author(s) -
PEREIRA C. M.,
COLETTA R. D.,
JORGE J.,
LOPES M. A.
Publication year - 2005
Publication title -
international journal of paediatric dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.183
H-Index - 62
eISSN - 1365-263X
pISSN - 0960-7439
DOI - 10.1111/j.1365-263x.2005.00627.x
Subject(s) - peutz–jeghers syndrome , medicine , mucocutaneous zone , dermatology , pathology , disease
Summary. 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.