[Multiple gastrointestinal familial poliposis (Peutz-Jerghers syndrome). Report of a clinical case].
The authors review a single case history of intestinal polyposis (Peutz-Jeghers syndrome) of a patient followed by them for eleven years. The paper includes a review of the pertinent literature. The present case was diagnosed as a result of the clinical observation of pigmented spots in the oral mucosa and crises of spasmodic abdominal pain accompanied by bowel movements containing mucus and blood. In other members of the family only a younger sister had slightly pigmented spots in the oral mucosa. None had radiologic findings suggestive of the syndrome of Peutz-Jeghers. Recently the patient under study suffered an intestinal intussuception which led to the surgical resection of a segment of small intestine and permitted pathologic confirmation of the diagnosis. Surgical resection of nodules in both breasts resulted in diagnoses of sclerosing adenosis in the left and an epidermoid cyst in the right.
D000328:Adult; D001706:Biopsy; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D007421:Intestine, Small / Q000000981:diagnostic imaging; D009061:Mouth Mucosa / Q000473:pathology; D009912:Oral Manifestations; D010580:Peutz-Jeghers Syndrome / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D010859:Pigmentation Disorders / Q000209:etiology; D011859:Radiography; D013270:Stomach / Q000000981:diagnostic imaging / Q000473:pathology
Polipósis familiar gastrointestinal múltiple (Síndrome de Peuts Jeghers). Presentación de un caso clínico.