Ehlers-Danlos syndrome is a group of disorders that affect connective tissues, which are tissues that support the skin, bones, blood vessels, and other organs. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos syndrome, which vary from mildly loose joints to life-threatening complications. People who have Ehlers-Danlos syndrome usually have overly flexible joints and stretchy, fragile skin. This can become a problem if you have a wound that requires stitches, because the skin often isn't strong enough to hold them. Ehlers–Danlos syndrome (EDS) is an inherited connective tissue disorder with different presentations that have been classified into several primary types.
The major signs and symptoms are of 4 types: Musculoskeletal, Skin, Cardiovascular, Other manifestations or complications The musculoskeletal symptoms are: Hyper-flexible joints, Thoracic outlet syndrome, Boutonniere deformity of the fingers, Unstable joints, Swan neck deformity, Tearing of tendons or muscles, Spine deformities, Myalgia and Arthralgia, Osgood-Schlatter Disease. Skin symptoms are: Fragile skin that tears easily, Easy bruising, Redundant skin folds, Molluscoid pseudotumors, Subcutaneous spheroids, Livedo reticularis. The cardiovascular symptoms are: Arterial rupture, Valvular heart disease, Dilation and/or rupture of ascending aorta, Postural orthostatic tachycardia syndrome, Raynaud's phenomenon, Varicose veins.
Worldwide, more than 1.5 million people are diagnosed with Ehlers-Danlos syndrome (EDS). EDS (all types combined) is reported to have a 1 in 5,000-10,000 population frequency. The population occurrence of EDS varies dependent on which 1 of the 6 major types according to the Villefranche nosology is diagnosed. EDS types and their population occurrence (from most to least common) are as follows: Hypermobility type EDS (type III) is the most common, occurring in 1 in 10,000-15,000 persons, Classical type EDS (types I and II) occurs in 1 in 20,000-50,000 persons, Vascular type EDS (type IV), considered the most serious type of EDS, is rare, occurring 1 in 100,000-250,000 persons, Kyphoscoliosis type EDS (type VI), arthrochalasia type EDS (type VII A and B), and dermatosparaxis type EDS (type VIIC) are all very rare, with dermatosparaxis type EDS being the rarest. Inferential and descriptive analyses were used in the presentation of the results.