The pigmented spots of Peutz-Jeghers syndrome can appear in which of the following ages?

The pigmented spots of Peutz-Jeghers syndrome can appear in which of the following ages?

• A. In childhood.
• B. Only in adulthood.
• C. Only after puberty.
• D. Not at all.

Answer: (A)

Pigmented spots in Peutz-Jeghers are mucocutaneous lentigines that most commonly appear in childhood, sometimes even at birth, around the lips, oral mucosa, and digits. This early-onset clue helps distinguish the syndrome, which is caused by a germline STK11 mutation and predisposes to hamartomatous GI polyps later on. While the spots persist throughout life, their appearance in childhood is the hallmark, making other timings—only in adulthood, only after puberty, or not at all—incorrect.

Pigmented spots in Peutz-Jeghers are mucocutaneous lentigines that most commonly appear in childhood, sometimes even at birth, around the lips, oral mucosa, and digits. This early-onset clue helps distinguish the syndrome, which is caused by a germline STK11 mutation and predisposes to hamartomatous GI polyps later on. While the spots persist throughout life, their appearance in childhood is the hallmark, making other timings—only in adulthood, only after puberty, or not at all—incorrect.