Peutz-Jeghers syndrome
- autosomal dominant (50% sporadic)
- hamartomas
- primarily of SB
- also: stomach, colon
- occasionally of urinary or respiratory tract
- mucocutaneous hyperpigmentation
- increased risk of GI + non-GI tumors! (NEJM 1988)
Cf: polyposis syndromes
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© 2002, C.E. Kahn, Jr.
2002-04-18