Peutz Jegher’s syndrome is a mucocutaneous autosomal dominant condition associated with extensive melanin pigmentation and intestinal hamartomatous polyp formation. It is characterised by round, oval patches of blackish brown pigmentation, irregularly distributed over the oral mucosa, lips, tongue and the hard palate. This condition is associated with an increased risk towards carcinoma and sev...

Among 72 patients with the Peutz-Jeghers syndrome malignant tumours have developed in 16 (22%) of whom all but one have died. There were nine gastrointestinal and seven nongastrointestinal tumours. The relative risks of death from gastrointestinal cancer and all cancers were 13 (95% CI 2.7-38.1) and 9 (95% CI 4.2-17.3) respectively. The chance of dying of cancer by the age of 57 was 48%. There ...

The paper describes a case of a 21-year-old woman admitted to the Department of Internal Medicine with signs and symptoms of microcytic anemia. The presence of characteristic skin lesions, results of laboratory tests and positive family history led to the diagnosis of a rare colonic polyposis, Peutz-Jeghers syndrome. The article presents also history, symptomatology, recommended tests and treat...

conclusions the patients with diffuse pjs may be asymptomatic and without gastrointestinal or extragastrointestinal malignancies. introduction peutz-jeghers syndrome (pjs) is a rare disorder characterized by mucocutaneous perioral pigmentation, gastrointestinal hamartomatous polyposis, and an increased risk of malignancy. families with pjs may show a variable spectrum of manifestations in spite...