Peutz Jeghers mucocutaneus

Peutz-Jeghers syndrome (PJS) is an inherited, autosomal dominant disorder characterised by haematomatous polyps in the gastrointestinal tract and mucocutaneus hyperpigmentation. A girl 15 years of age presented with microcytic, hypochrome anaemia. Endoscopy revealed several polyps in the stomach and colon. The patient was later operated due to an intussusception from polyps in the jejunum. Histology proved the polyps to be haematomatous as seen in PJS. Patients with PJS have an increased risk of developing both gastrointestinal and extra-gastrointestinal cancers...

Peutz-Jeghers' syndrome (PJS) is an autosomal dominant inherited disease, which is characterized by mucocutaneus pigmentation and hamartomatous gastro-intestinal polyps. Intussusceptions can easily occur in patients with PJS. Repeated laparotomy with resections and eventual short bowel syndrome is a major problem in these patients. We present a 20-year-old woman with multiple intestinal intussusceptions. Multiple enterotomies and intestinal resections were performed to achieve a longer symptom-free period.

Peutz-Jeghers syndrome is an autosomal dominant inherited disorder which is characterized by mucocutaneus melanocytic macules and intestinal hamartomatous polypus. The aim of our work is to underline the role of pigmented changes in the diagnostic process. Numerous lentigines should always lead to multiorgan investigations.