What syndrome is characterized by hamartomatous gastrointestinal polyps and hyperpigmentation of the mouth, feet, and hands?

Peutz-Jeghers syndrome is characterized by the presence of hamartomatous polyps in the gastrointestinal tract, particularly in the small intestine, and distinctive skin findings, including hyperpigmentation of the lips, mouth, hands, and feet. This syndrome is caused by mutations in the STK11 gene.

The gastrointestinal polyps in Peutz-Jeghers syndrome are non-cancerous but can lead to complications such as intestinal obstruction due to their ability to grow large. Furthermore, the associated mucocutaneous pigmentation, often noticed from an early age, is a hallmark of the condition and is most prominently seen on the labial mucosa and fingertips.

In summary, Peutz-Jeghers syndrome combines these two significant features: hamartomatous polyps, primarily in the gastrointestinal tract, and specific pigmentation changes in the skin and mucosal areas, which clearly delineates it from other syndromes such as Gardner's syndrome, familial adenomatous polyposis, and Cowden syndrome, each of which presents with different types of polyps or distinct clinical features.