When do the characteristic skin lesions of Peutz-Jeghers syndrome appear? Brown to blue-black macules (lentigines) are present at birth or early infancy on the lips, oral mucosa, nasal mucosa, palms, soles, dorsal hand surfaces, central face, and elbows. Polyps of the small intestine develop in 90% of patients; polyps may also occur in the stomach, colon, and rectum. There is an increased incidence of gastrointestinal malignancy, but it is not nearly as common as in patients with Gardner’s syndrome. In one series, 16 fatal malignancies were seen in 72 cases, with an average age at death of 36 years. Intussusception occurs in about 50% of cases. |
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