Peutz-Jeghers Syndrome

**Definition:** A hereditary disease caused by autosomal dominant mutations involving CHROMOSOME 19. It is characterized by the presence of INTESTINAL POLYPS, consistently in the JEJUNUM, and mucocutaneous pigmentation with MELANIN spots of the lips, buccal MUCOSA, and digits.

**Tree numbers:** - C04.700.633 - C06.405.469.578.750 - C16.320.700.667 - C17.800.621.430.530.550.625

**Synonyms:** - Lentiginosis, Perioral - Polyposis, Hamartomatous Intestinal - Polyps-and-Spots Syndrome - Peutz-Jeghers Polyposis - Periorificial Lentiginosis Syndrome - Peutz-Jegher's Syndrome