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Peutz–Jeghers Syndrome: Gastrointestinal Features

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Atlas of Dermatological Manifestations of Gastrointestinal Disease

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Abstract

Peutz–Jeghers syndrome is a genetic disease characterized by the development of benign hamartomatous polyps in the gastrointestinal (GI) tract and macules on the lips and oral mucosa. The incidence rate is not clear.

GI symptoms include:

Bleeding, anemia, abdominal pain

More severe: intussusception, obstruction, infarct

Clinical signs and findings should include :

Characteristic “hamartoma” polyps are found in the small bowel (90 %) and colon (50 %)

Symptoms from polyps arise in 33 % of patients by age 10 years and 50 % by age 20 years

Mucocutaneous hyperpigmented macules that develop in early childhood

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References

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Author information

Authors and Affiliations

  1. Yale Department of Internal Medicine, Yale New Haven Hospital, 20 Yale street, New Haven, CT, 06510, USA

    Liam Zakko

Authors
  1. Liam Zakko
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Corresponding author

Correspondence to Liam Zakko .

Editor information

Editors and Affiliations

  1. Department of Medicine, Gastroenterology Division,, University of Connecticut Health Center, 263 Farmington Ave., Farmington, 06030, Connecticut, USA

    George Y. Wu

  2. , Department of Medicine, University of Connecticut, 56 Kingswood Road, West Hartford, 06119, Connecticut, USA

    Nathan Selsky

  3. , Univ. of CT Health Ctr, Dermatol. Dept., University of Connecticut, 21 South Road, Farmington, 06032, Connecticut, USA

    Jane M. Grant-Kels

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Zakko, L. (2013). Peutz–Jeghers Syndrome: Gastrointestinal Features. In: Wu, G., Selsky, N., Grant-Kels, J. (eds) Atlas of Dermatological Manifestations of Gastrointestinal Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6191-3_35

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  • DOI: https://doi.org/10.1007/978-1-4614-6191-3_35

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6190-6

  • Online ISBN: 978-1-4614-6191-3

  • eBook Packages: MedicineMedicine (R0)

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