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Howel-Evans Syndrome: Dermatological Features (Familial Keratoderma with Carcinoma of the Esophagus)

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Atlas of Dermatological Manifestations of Gastrointestinal Disease

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Abstract

Clinical signs and features include:

Genodermatosis associated with carcinoma of the esophagus

Leukoplakia

Palmoplantar keratoderma (PPK): thickening of the skin on the palms and soles with pruritus and deep, painful fissures; focal hyperkeratotic skin related to physical activity and with regression on bed rest

PPK begins in the second decade

Associated risk of esophageal squamous cell cancer with 90 % developing by age 65 and usually earlier at age 45

Two families in the United Kingdom and one family in the United States have been found with the condition to date

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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 York Street, New Haven, CT, 06510, USA

    Liam Zakko

  2. Department of Dermatology, University of Connecticut Health Center, 21 South Road, Farmington, CT, 06030, USA

    Justin Finch, Marti J. Rothe & Jane M. Grant-Kels

Authors
  1. Liam Zakko
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  2. Justin Finch
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  3. Marti J. Rothe
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  4. Jane M. Grant-Kels
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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., Finch, J., Rothe, M.J., Grant-Kels, J.M. (2013). Howel-Evans Syndrome: Dermatological Features (Familial Keratoderma with Carcinoma of the Esophagus). 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_6

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  • DOI: https://doi.org/10.1007/978-1-4614-6191-3_6

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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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