Ehlers-Danlos Syndrome (EDS) describes a group of approximately eleven hereditary disorders mainly affecting the connective tissues from various parts of the body, such as skin, muscles, tendons, blood vessels, and ligaments. EDS can also affect other organs. In EDS, collagen, a common protein found in many types of connective tissues, is defective, leading to abnormalities in various connective tissues and subsequently to a variety of signs and symptoms (see below). Each type of EDS is distinct. In fact, individuals with the same type of EDS may have slightly different manifestations from each other.
EDS is estimated to occur in approximately 1 in 5000 births, although the true incidence of this disease is unknown because it is difficult to diagnose EDS.
Physicians who more commonly diagnose EDS include medical geneticists, pediatricians, rheumatologists and dermatologists.
Causes
EDS is caused by various defects in collagen, the main structural protein in the body. In some parts of the body, collagen provides strength and support. In other parts of the body, collagen functions to provide elasticity to allow movement and glue to allow binding of proteins to one another. Consequently, if collagen is defective, it can produce a variety of problems.Risk Factors
Surgical procedures can be risky, as fragile tissues can unexpectedly tear. If you are scheduled for any type of surgery, be sure to tell the surgeons if you have Ehlers-Danlos Syndrome. You should also tell your surgeon if you have any vascular or bleeding problems.
Last updated:
Jan-01-09
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