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Atlas of Dermatological Manifestations of Gastrointestinal Disease pp 75–76Cite as

Ehlers–Danlos Syndrome Type IV (Vascular): Dermatological Features

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Abstract

Clinical signs and features include:

In general, Ehlers–Danlos syndrome is characterized by skin hyperextensibility, joint hypermobility, and wound healing abnormalities

Type IV (vascular) Ehlers-Danlos is the type most commonly associated with gastrointestinal (GI) pathology

Type IV has less skin hyperextensibility. The major cutaneous finding is very translucent skin with easily visible veins particularly on the chest, abdomen, and extremities. The most common, and often the first sign of the disease, is pronounced bruising.

Series of patients show that 7–25 % of patients experience the first major complication by age 20 years and more than 75 % by age 40 years.

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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

  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). Ehlers–Danlos Syndrome Type IV (Vascular): Dermatological 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_30

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  • DOI: https://doi.org/10.1007/978-1-4614-6191-3_30

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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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