A Case of Peutz-Jeghers Syndrome with Nasal Polyposis / 대한내과학회지

ABSTRACT

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disease characterized by typical mucocutaneous pigmentation and multiple hamartomatous polyps in the gastrointestinal tract. The clinical manifestations of PJS include polyp-induced bowel obstruction, abdominal pain, and gastrointestinal bleeding. The risk of gastrointestinal and extragastrointestinal cancers is also significantly increased in patients with PJS. Nasal polyposis associated with PJS has been described, and is considered an extragastrointestinal manifestation of PJS. We present an unusual case of a patient with PJS and nasal polyposis causing complete unilateral nasal obstructio.