Sunday, October 9, 2016

Peutz–Jeghers syndrome



Which of the following statements about Peutz–Jeghers syndrome is true?

A) The condition is sex linked and usually skips a generation.
B) The condition involves the development of multiple polyps in the stomach and the small and large intestine that commonly show malignant change.
C) There is associated hyperpigmentation around the oral cavity lips, soles of the feet, and dorsum of the hands.
D) The condition is associated with inflammatory bowel disease.
E) The condition is identified by elevation in carcinoembryonic antigen levels.

Answer and Discussion

The answer is C. There is associated hyperpigmentation around the oral cavity lips, soles of the feet, and dorsum of the hands.

 Peutz–Jeghers syndrome is a familial autosomal dominant condition that involves the development of multiple, benign, hamartomatous polyps in the stomach and in the small and large intestine.
Malignant change has occurred but is rare. Those affected also have melanin-associated brownish-black hyperpigmentation around the oral cavity, lips, soles of the feet, and dorsum of the hands. The condition usually causes no problems except in severe cases in which abdominal pain, intestinal obstruction, or bleeding can occur. In these severe cases, surgery may be considered

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