Peutz-Jeghers syndrome is an autosomal dominant disorder characterised by mucosal pigmentation of the lips and gums with multiple intestinal hamartomatous polyps.There is an associated marked increased risk of certain malignancies, especially gastrointestinal (GI) cancers and breast cancer.Typically, signs of this condition appear in childhood with the development of pigmented areas on the skin and in the mouth (called mucocutaneous hyperpigmentation). People with PJS tend to develop dark blue or dark brown freckling, especially around the mouth and on the lips, fingers, or toes.
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