Why is it important to identify patients with Peutz-Jeghers syndrome?

Patients with Peutz-Jeghers syndrome have widespread cutaneous lentigines that involve the arms, legs, torso, digits, lips, buccal mucosa, palate, tongue, and eyelids. In addition, they suffer from gastrointestinal (GI) polyps, usually in the small bowel, which, by the second decade of life, can become symptomatic with diarrhea, hemorrhage, obstruction, or intussusception. Malignant degeneration of GI polyps has been reported, with carcinomas developing most commonly in the large intestine but also in the small intestine and stomach.