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Diseases of Pigment

Picture of Peutz-Jeghers Syndrome

Picture of Peutz-Jeghers Syndrome

Peutz-Jeghers syndrome is a rare genetic disorder that causes small spots on the mouth, fingers, and lips. The condition also causes the formation of polyps in the intestines. The syndrome substantially increases the risk of cancers of the stomach, rectum, esophagus, breast, ovaries, pancreas, testes, and colon. The middle part of the small intestine, the jejunum, is most often affected by polyps. The inheritance of Peutz-Jeghers syndrome is in an autosomal dominant pattern. A mutation in a tumor suppressor gene called STK11 causes the condition.

Image Source: Color Atlas & Synopsis of Pediatric Dermatology Kay Shou-Mei Kane, Jen Bissonette Ryder, Richard Allen Johnson, Howard P. Baden, Alexander Stratigos Copyright 2002 by The McGraw-Hill Companies. All rights reserved.

Text Reference: Genetics Home Reference: "Peutz Jeghers"