Abstract
Ehlers–Danlos syndrome is a group of inherited connective tissue disorders caused by a defect in the synthesis of collagen. Severity of the disease depends on the mutation inherited. The disorder occurs in approximately one in 5,000 births.
Gastrointestinal (GI) signs and findings include:
Epigastric discomfort, hematemesis, melena, abdominal pain, constipation, peritoneal signs
In general, Ehlers–Danlos Syndrome is characterized by skin hyperextensibility, joint hypermobility, and wound-healing abnormalities
Type IV (vascular) Ehlers–Danlos is most often associated with GI pathology
Type IV has less skin hyperextensibility; major skin finding is very translucent skin with easily visible veins (particularly on the chest); thin faces, pinched nose, and large eyes
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Zakko, L. (2013). Ehlers–Danlos Syndrome Type IV (Vascular): 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_29
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DOI: https://doi.org/10.1007/978-1-4614-6191-3_29
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