Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant (AD), inherited condition characterized by the development of unique benign hamartomatous polyps throughout the gastrointes...
PJS may be discovered incidentally usually by suggestive mucosal hyperpigmentation or by family history.
Half of patients affected by PJS presented by age 20 years with recurrent abdom...
Management primarily involves minimizing symptoms by screening for long-term complications (surveillance).
Surgical strategies are common for mana...
Q85.8 Other phakomatoses, not elsewhere classified
K31.7 Polyp of stomach and duodenum
K63.5 Polyp of colon
54411001 Peutz-Jeghers syndrome (disorder)
53633000 Peutz-Jeghers polyps of sma...
Consider hereditary polyposis syndromes in the following:
Cancer at very young age compared with usual presentation
Presence of multiple tumors, especially hamartomas
Family history of si...
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Peutz-Jeghers Syndrome When pigmented spots on the lips are more prominent than freckling of the surrounding skin, suspect this syndrome. Pigment in the buccal mucosa helps to confirm the diagnosis. Pigmented spots may also be found on the face and hands. Multiple intestinal polyps are often associated.
Peutz-Jeghers Syndrome When pigmented spots on the lips are more prominent than freckling of the surrounding skin, suspect this syndrome....