# Peutz-Jeghers syndrome

INSERM

## Source

INSERM. (1999). Orphanet: an online rare disease and orphan drug data base. *Peutz-Jeghers syndrome*. ORPHA:2869

Peutz-Jeghers syndrome (PJS) is an inherited gastrointestinal disorder characterized by development of characteristic hamartomatous polyps throughout the gastrointestinal (GI) tract, and by mucocutaneous pigmentation. PJS carries a considerably increased risk of GI and extra-GI malignancies.