Peutz-Jeghers syndrome

Alternative names:

familial adenomatous polyposis

Definition:

An inherited disorder characterized by intestinal polyps and pigmentation of the mucus membranes and skin.

Causes, incidence, and risk factors:

Peutz-Jeghers syndrome is transmitted as an autosomal dominant trait. Pigmented spots, brownish or bluish gray, develop from infancy through childhood around the lips, gums, and mucus membranes in the mouth.

Intestinal polyps also develop, but these cannot be seen without special studies. These may cause abdominal cramping, intussusception, chronic bleeding, and anemia from the chronic blood loss. Polyps can also become malignant.