What are the cutaneous signs of Peutz-Jeghers syndrome (PJS)?

Updated: Oct 11, 2018
  • Author: Buu Anh T To, MD; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Answer

Hyperpigmented mucocutaneous macules may be present on the lips and buccal mucosa and around the mouth, eyes, and nostrils, as well as sparsely on the fingers, soles of the feet, palms, anal area, and intestinal mucosa. [2, 15]  The macules are flat, blue-gray to brown-black spots 2-4 mm in size. They tend to develop by age 5 years but are rarely present at birth. Some may fade during the onset of puberty, but buccal mucosa lesions tend to persist. [2, 12, 13, 15, 59, 72, 73]

See the images below.

This is a facial photograph of a patient with Peut This is a facial photograph of a patient with Peutz-Jeghers syndrome. Note the mucocutaneous pigmentation that crosses the vermilion border.
This photograph shows an oral pigmented lesion in This photograph shows an oral pigmented lesion in a patient with Peutz-Jeghers syndrome.

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