Peutz-Jeghers Syndrome: A rare genetic disorder characterized by the development of numerous benign nodules on the inside lining of the intestinal wall as well as excess skin pigmentation usually around the lips and inner lining of the mouth. More detailed information about the symptoms, causes, and treatments of Peutz-Jeghers Syndrome is available below.
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An inherited condition characterized by generalized hamartomatous multiple polyposis of the intestinal tract. Transmitted in an autosomal dominant fashion, Peutz-Jeghers syndrome consistently involves the jejunum and is associated with melanin spots of the lips, buccal mucosa, and fingers. This syndrome is associated with abnormalities of chromosome 19. Also known as Jeghers-Peutz syndrome and Peutz's syndrome. --2004 - (Source - Diseases Database)
Peutz-Jeghers Syndrome is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Peutz-Jeghers Syndrome, or a subtype of Peutz-Jeghers Syndrome,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Peutz-Jeghers Syndrome as a "rare disease".
Source - Orphanet
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