Different Types of Ehlers-Danlos Syndromes

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Ehlers-Danlos syndrome (EDS) is a collection of inherited conditions that fit into a larger group, known as heritable disorders of connective tissue.

Connective tissues provide support in skin, tendons, ligaments, blood vessels, internal organs and bones. Listed are the major types of EDS, though, rarer types have been discovered. (They used to be classified by numbers: Type I, Type II, etc. Since 1988, they are referred more now to their name.)

 



The Hypermobility Type of EDS is characterized by loose joints and chronic joint pain.


Vascular Type

The Vascular Type of EDS is characterized by possible arterial or organ rupture as a result of spontaneous rupture of vessels or organs due to the result of even minor trauma.  The Vascular Type of EDS is the most serious form of Ehlers-Danlos Syndrome.

People with vascular EDS may have: skin that bruises very easily because of fragile tissues, thin skin with visible small blood vessels, particularly on the upper chest and legs, fragile blood vessels – this can lead to major complications, such as blood vessels tearing (dissection) and arterial aneurysms (artery widening with a risk of rupture), risk of damage to hollow organs, such as bowel perforation or uterine rupture (where the womb tears),Occasionally there may be other features including: hypermobility of small joints (such as fingers and toes), premature ageing of the skin on hands and feet, unusual facial features, such as a thin nose and lips, large eyes, small earlobes and fine hair, joint contractures (permanent shortening of a joint), partial collapse of the lung (pneumothorax), gum problems, such as bleeding or receding gums, varicose veins in early adult life, wounds may take longer to heal


 

Kyphoscoliosis Type


The Kyphoscoliosis Type of EDS is characterized by generalized joint laxity and severe muscle hypotonia (weak muscle tone) at birth. 

People with kyphoscoliotic EDS may have: curvature of the spine (scoliosis) – this starts in early childhood and often gets worse in the teenage years, loose, unstable joints that frequently lead to dislocations, weak muscle tone from childhood – this may cause a delay in sitting and walking, or difficulty walking if symptoms progress, fragile eyeballs that can easily be damaged, unusual shape or size of the clear front part of the eye (cornea), soft, velvety skin that is stretchy, bruises easily and scars


 

Arthrochalasia Type


 

Dermatosparaxis Type

The Dermatosparaxis Type of EDS is characterized by severe skin fragility and substantial bruising. Wound healing is not impaired and the scars are not atrophic. The skin texture is soft and doughy. Sagging, redundant skin is evident. The redundancy of facial skin results in an appearance resembling cutis laxa. Large hernias(umbilical, inguinal) may also be seen. The number of patients reported with this type of EDS is small.


Tenascin-X Deficient Type


It’s also important to understand that even if you have a certain type of EDS, it does not mean that you do not have Vascular problems. One can even have a mutated gene from one parent combined with another mutated gene from another parent and have both types. There is a 50% chance of even passing the mutated gene on.
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