Patta-Jeghers syndrome

Patta-Jeghers syndrome (J. L. Peutz, N. Jeghers; synonym syndrome Patta - Touraine) is a syndrome characterized by a combination of pigment spots on the skin and mucous membranes and polyps in the gastrointestinal tract. Age spots (see Lentigo) dark-brown, round (or oval shape, with a diameter from 1 to 5 mm, are usually on the skin around mouth and eyes, nose), sometimes on the limbs, on the mucous membrane of the mouth (and sometimes the genitals and rectum). Polyps often observe in the small intestines (sometimes in the nose and urinary tract). Patients complain of pain in the abdomen, constipation, frequent bleeding and bowel obstruction. In 20% of cases observed malignant degeneration.
Pigmentation appears in early childhood, and is often hereditary. Polyposis occurs between the ages of 5 and 30 years. The disease often a family nature. Sick in an equal measure both sexes. The etiology is unknown. Some authors stress the similarity with abortive form of neurofibromatosis.
During hemorrhage, intestinal obstruction, severe pain showed surgery.