A young woman with mucocutaneous pigmentation and intestinal polyps.

A 26-year-old woman was regularly followed up in a gastro-intestinal out-patient clinic. Since early childhood, she was noted to have hyperpigmented lesions over the perioral region (Fig 1a) and fingers (Fig 1b), and a history of small intestinal intussusception due to polyps that was treated by surgery when she was 12 years old. Endoscopic assessment revealed multiple gastric polyps (Fig 1c) and a colonic hamartoma (Fig 1d). The family history was otherwise unremarkable for gastro-intestinal disease or malignancy.