Peutz-Jeghers syndrome history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]
Overview
Symptoms of Peutz-Jeghers syndrome include mucocutaneous hyperpigmentation, rectal bleeding, abdominal pain, and weight loss.
History and Symptoms
Symptoms of Peutz-Jeghers syndrome include the following:
- Mucocutaneous hyperpigmentation
- Rectal bleeding
- Abdominal pain
- Weight loss
The risks associated with this syndrome include a strong tendency of developing cancer in multiple sites[1]. While the harmartomatous polyps themselves do not have malignant potential, patients with the syndrome have an increased risk of developing carcinomas of the pancreas, liver, lungs, breast, ovaries, uterus and testicles.
The average age of first diagnosis is 23, but the lesions can be identified at birth by an astute pediatrician. Prior to puberty, the mucocutaneous lesions can be found on the palms and soles. Often the first presentation is as a bowel obstruction from an intussusception which is a common cause of mortality; an intussusception is a telescoping of one loop of bowel into another segment.
Most of the data regarding this disorder are from selected family lines and thus the risks endured by those families regarding outcomes may not translate completely to the patient without a familial history.