Unusual manifestations of the Peutz-Jeghers syndrome.

گزارش یک مورد سندرم پوتزژوگر Peutz Jeghers Syndrome

[Peutz-Jeghers syndrome].

Peutz-Jeghers syndrome (PJS, MIM 175,2000) is a disease of autosomal dominant inheritance that is characterised by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. In addition to problems such as intussusception, PJS predisposes to cancers of several sites. The unusual combination of clinical features makes the identification of the defect underlying PJS particularly intere...

Laugier-Hunziker syndrome: an important differential diagnosis for Peutz-Jeghers syndrome.

Laugier-Hunziker syndrome (LHS) is a rare sporadic disorder, which shares some dermatological features with Peutz-Jeghers syndrome (PJS). However, whereas PJS is associated with hamartomatous gastrointestinal polyposis and carries a high risk of malignancy justifying intensive screening protocols, LHS is known to be an entirely benign disease with no systemic manifestations, which requires pati...

Peutz-Jeghers syndrome: a new understanding.

Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by hamartomatous polyps in the small bowel and mucocutaneous pigmentation. Patients with Peutz-Jeghers syndrome often present as surgical emergencies with complications of the polyps, such as intussusception, bowel obstruction and bleeding. Furthermore, repeated operations may be needed in some patients, which may ...

Unusual presentation of intussusception of the small bowel with peutz jeghers syndrome: report of a case.

The Peutz Jeghers syndrome (PJS) is an autosomal dominant disorder which is characterised by hamartomatous polyposes of the gastrointestinal tract, melanin pigmentation of the skin and mucous membranes, and an increased risk for cancer. We are reporting a case of a 15-year-old male with Peutz Jeghers syndrome, who presented to us with features of chronic intestinal obstruction and anaemia. Init...