The mucocutaneous lesions of PJS consist of hyperpigmented (dark brown to black), round, 1 to 5 mm macules distributed on the central part of the face, lips, and oral mucosa (Fig. 5). Hyperpigmented macules may also be seen on the dorsal hands, feet, perianal area,
and periumbilical skin. The macules can be present at birth or arise early in life. The cutaneous lesions can fade with time, but the mucosal hyperpigmentation remains. The hyperpigmented macules in PJS can be distinguished from freckles by their mucosal involvement and the fact that they are commonly present at birth. The mucocutaneous lesions in PJS precede the gastrointestinal manifestations. The median time to presentation with polyps is 11 years of age, but the age of onset varies greatly. There are reported cases of patients presenting with polyposis at birth, but this is very rare. Extraintestinal polyposis may develop and can involve the nares, pelvis, bladder, and lungs. Although the rate of neoplastic potential of the intestinal lesions is low, 48% of patients with PJS have been reported to develop intestinal and extraintestinal malignancies that include neoplasms of the colon, esophagus, rectum, stomach, small intestine, breast, pancreas, ovaries, cervix, testicles, and uterus.
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