Drawing from 1896 publication of twins thought to have<unk>  The main criteria for clinical diagnosis are: * Family history * Mucocutaneous lesions causing patches of hyperpigmentation in the mouth and on the hands and feet. The oral pigmentations are the first on the body to appear, and thus play an important part in early diagnosis. Intraorally, they are most frequently seen on the gingiva, hard palate and inside of the cheek. The mucosa of the lower lip is almost invariably involved as well. * Hamartomatous polyps in the gastrointestinal tract. These are benign polyps with an extraordinarily low potential for malignancy. Having 2 of the 3 listed clinical criteria indicates a positive diagnosis. The oral findings are consistent with other conditions, such as Addison's disease and McCune–Albright syndrome, and these should be included in the differential diagnosis. 90–100% of patients with a clinical diagnosis of PJS have a mutation in the STK11/LKB1 gene. Molecular genetic testing for this mutation is available clinically.
Peutz–Jeghers syndrome
Juvenile polyp
Familial adenomatous polyposis
Nijmegen breakage syndrome
MUTYH-associated polyposis
Juvenile polyposis syndrome
Serrated polyposis syndrome
Li–Fraumeni syndrome