Peutz-touraine syndrome (peutz-jeghers syndrome)

General: Recognized in infants; autosomal dominant; gastrointestinal polyps; jejunal polyps are consiste 111b17b nt feature; these lesions are benign, melanin spots of the lips; buccal mucosa and digits represent second part of syndrome.

Ocular: Brownish speckled dots along border of skin and mucosa of eyelids; freckles similar to those seen on eyelids may appear on conjunctiva, primarily adjacent to limbus and along area of lid fissure; pigment speckles of sclera and iris; brown-pigmented corneal spot.

Clinical: Brownish to black dotlike pigmentation similar to that seen on eyelids is present in the perinasal, perioral, and periorbital regions, fewer on fingers and toes at birth; polyposis of gastrointestinal tract (primarily small intestine) with associated bleeding; potential for malignant transformation in second decade of life.

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