Peutz-Jeghers Syndrome is a dominantly inherited disorder characterized by mucocutaneous pigmentation and gastrointestinal polyposis. Peutz-Jeghers polyps may occur anywhere in the intestine and can become malignant. Patchy circumoral pigmentation is characteristic, and may not be as obvious as in this example. The pigmentation should always be sought carefully in young patients presenting with unexplained gastrointestinal bleeding, particularly if there is a family history of such bleeding.
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