Peutz-Jeghers Syndrome

Peutz-Jeghers Syndrome. Freckle-like lips and all around the buccal mucosa.


Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with mucocutaneous melanocytic macules. Although the intestinal lesions are hamartomas, patients with Peutz-Jeghers syndrome have a 15-fold increased risk of developing intestinal cancer compared to that of the general population. Cancer location includes gastrointestinal and extraintestinal sites.

The gastrointestinal polyps found in Peutz-Jeghers syndrome are typical hamartomas. Their histology is characterized by extensive smooth muscle arborization throughout the polyp. This may give the lesion the appearance of pseudoinvasion, because some of the epithelial cells, usually from benign glands, are surrounded by the smooth muscle. The risks associated with this syndrome include a strong tendency of developing cancer in GIT and other areas include the pancreas, liver, lungs, breast, ovaries, and testicles.

The Criteria:
Family history of Peutz-Jeghers syndrome
Repeated bouts of abdominal pain in patients younger than 25 years
Unexplained intestinal bleeding in a young patient
Prolapse of tissue from the rectum
Menstrual irregularities in females (due to hyperestrogenism from sex cord tumors with annular tubules)
Gynecomastia in males (possible due to the production of estrogens from Sertoli cell testicular tumors)
Precocious puberty
Gastrointestinal intussusception with bowel obstruction(prolapse of ileum to caecum)


Caffeine,My Morphine.
Daily Intake Increased.

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