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

  • Liam Zakko
  • Justin Finch
  • Marti J. Rothe
  • Jane M. Grant-Kels


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.


Marfan Syndrome Autosomal Dominant Disease Joint Hypermobility Foreign Body Giant Cell Gingival Recession 
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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Liam Zakko
    • 1
  • Justin Finch
    • 2
  • Marti J. Rothe
    • 2
  • Jane M. Grant-Kels
    • 2
  1. 1.Yale Department of Internal MedicineYale New Haven HospitalNew HavenUSA
  2. 2.Department of DermatologyUniversity of Connecticut Health CenterFarmingtonUSA

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