Resumo (EN)
Peutz-Jeghers syndrome is an autosomal dominant condition characterized by the association of gastrointestinal polyps associated with mucocutaneous pigmentation and increased pre-disposition to neoplasms in multiple organs, especially at the level of the small intestine. It is a rare condition, having a prevalence of approximately 1 in 100,000 to 200,000 births. It was first recognized in 1921 by Peutz in a Dutch family. Hyperpigmentation is present as mucocutaneous macules on the lips and around the mouth, eyes, nose, and on the buccal mucosa, palms and dorsum of the hands, fingers and soles of the feet, tending to disappear at puberty, but the oral mucosa pigmentation remains. The treatment of lentigines represents a cosmetic problem for its patients. These polyps are also at risk of acute gastrointestinal bleeding, intussusception and intestinal obstruction. Regular surveillance is recommended since the first decade of life. This paper reports a case of Peutz-Jeghers syndrome with no family history.