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Peutz-Jeghers syndrome (PJS) is characterised by the association of hamartomatous digestive polyposis and mucocutaneous lentiginosis. Patients are at risk of mechanical and haemorrhagic complications. It is a syndrome predisposing to cancer. We report the case of a 44-year-old man with a history of brown pigment stains around the mouth at the age of 10, which disappeared in adulthood, followed by bowel resections at 3, 16 and 18 years of age for occlusive episodes, having presented a month ago with diffuse abdominal distension complicated within three days by an occlusive syndrome with abdominal pain of moderate intensity, with no other associated digestive signs including digestive or extra digestive haemorrhage, all evolving in a context of altered general condition with weight loss. On clinical examination, the flanks were dull, there was diffuse abdominal tenderness and a median laparotomy scar. The patient underwent FOGD and colonoscopy, which revealed a mucosa lined with gastro-duodenal and colonic polyps of varying size and suspicious appearance. A biopsy of these polypoid formations was performed, confirming Peutz-Jeghers syndrome. A cytological and immunohistochemical study of the ascites fluid found atypical carcinomatous cells. In the literature, Peutz-Jeghers syndrome may be revealed clinically or at the stage of complications such as haemorrhage, intussusception or intestinal obstruction. As in the case of our patient, who presented with an occlusive syndrome with no detectable digestive haemorrhage. Peutz-Jeghers syndrome is a rare condition. However, it is important for clinicians to be aware of it and to consider its possibility in cases of occlusive syndrome with peri-orificial lentiginosis in young adults.
Outaghyame et al. (Fri,) studied this question.