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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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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