A. In patients without a family history of PJS, one of the following must be present: | • Characteristic melanotic macules and one or more intestinal polyps with PJS-type histology*, or |
• Two intestinal polyps with PJS-type histology* | |
B. In patients with a family history of PJS in a first degree relative, one of the following must be present: | • Characteristic melanotic macules, or |
• One intestinal polyp with PJS-type histology*, or | |
• STK11 mutation |