Oral Pigmentation and Intestinal Polyposis

IN 1921 Peutz described a family with intestinal polyposis and spots of melanin pigmentation in and around the mouth. Prior to this, in 1896, Sir Jonathan Hutchinson described two girls with pigmented patches in the oral mucosa and around the mouth. Later, Parkes Weber (1919) reported that one of these twins died of what appeared to be an intussusception. Since 1921 over one hundred cases have been described. The syndrome was first recognised in the British Isles by Tanner in 1951. In 1959 Falkinburgh and Kay reviewed the literature up to that date. The Peutz-Jeghers syndrome is a rare but well-defined syndrome. In view of the serious consequences, despite the ease of diagnosis, a further case is worth reporting.