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Peutz-Jegher's syndrome

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Peutz-Jegher's syndrome is an autosomal dominant condition characterised by:

  • multiple hamartogenous polyps of the gastrointestinal tract - most often in the small bowel but may occur affect any portion of the GI tract
  • mucocutaneous pigmentation - mainly, of the lips, buccal mucosa, genitalia, hands and feet

Patients often present with small bowel intussusception before the age of 10 years.

The polyps themselves have a very low malignant potential. About 10-20% of patients develop gastrointestinal carcinoma but this is thought to arise from coexistent adenomas.

Patients have an increased risk of developing carcinomas of the pancreas, lung, ovary and breast.

Click here for an example image of this condition

 


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