PEUTZ-JEGHERS SYNDROME

By Gary M. White, MD

Peutz-Jeghers syndrome


Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the association of mucocutaneous pigmentation and multiple gastrointestinal hamartomatous polyps.

Clinical

Pigmented macules develop in infancy or early childhood on the lips and buccal mucosa. Pigmented macules of the palms, fingers and soles also occur.

Treatment

The majority of the care required is provided by the gastroenterologist. Routine monitoring and removal of larger or symptomatic polyps has been recommended. Routine surveillance for non-GI malignancies should also be done. The Q-switched ruby laser is effective for the lentigines.

Additional Pictures

Peutz-Jeghers syndrome Peutz-Jeghers syndrome

Peutz-Jeghers syndrome Peutz-Jeghers syndrome

Peutz-Jeghers syndrome Peutz-Jeghers syndrome

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