Repeated episodes of abdominal pain in persons having melanin spots with a characteristic distribution can, with strong suspicion, denote existent intestinal polyps. Typically the melanin spots are on not only the lips and labial mucous membrane but also the buccal mucosa, occasionally the gingiva and hard palate, and rarely on the tongue. The pigmentation can also occur on the digits, periorally, about the eyes, and more rarely across the bridge of the nose. The condition has been reported in persons from childhood to the sixth decade, but usually appears as a recurrent intussusception in the second decade. It is to be emphasized that the polyps in this syndrome are to be considered premalignant lesions.
Rohrs LC. INTESTINAL POLYPOSIS AND PIGMENTED SPOTS OF LIPSHEREDITARY PEUTZ-JEGHERS SYNDROME. JAMA. 1957;165(3):208–210. doi:10.1001/jama.1957.02980210004002
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