Rare Zebra

Rare disease search prototype built on Orphanet data

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ORPHA:2869

Peutz-Jeghers syndrome

Also called Hamartomatous intestinal polyposis, PJS

Body system
Skin diseases
Inheritance pattern
Autosomal dominant
Typical age of onset
Adolescent, Adult, Childhood
Estimated prevalence
1-9 / 100 000 (Worldwide)
Rarity class
1-9 / 100 000

ORPHA:2869 is classified under "Skin diseases" in the Orphanet nomenclature.

Common signs

  • Multiple lentigines
  • Gastrointestinal carcinoma
  • Abnormality of the gastrointestinal tract
  • Macule
  • Abnormal pigmentation of the oral mucosa
  • Gastrointestinal hemorrhage