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Ehlers Danlos Syndrome Overview


Types of Ehlers-Danlos Syndrome

Although all forms of Ehlers-Danlos Syndrome share certain symptoms, such as muscle fatigue and stretchy skin, the six distinct types of EDS each present specific symptoms. Three of these types are extremely rare.

The most common forms of EDS are hypermobility, classical EDS and vascular EDS. Hypermobility, which affects 1 in 10,000 to 15,000 people, causes looseness in both large and small joints. Dislocations are common, and patients often experience pain in their joints and limbs [source: Mayo Clinic]. Classical EDS affects 1 in 20,000 to 40,000 people, and it's marked by skin hyperextensibility (stretchiness), scars and wounds that don't heal properly, fatty and noncancerous growths, and delicate skin tissue that is vulnerable to bruising [source: Mayo Clinic]. Vascular EDS has been reported in an estimated 1 in 250,000 people. This type is considered highly serious because of the risk of major organs or blood vessels rupturing. People with vascular EDS have very thin and fragile skin, which is often so translucent that veins can be seen through it [source: Mayo Clinic]. Easy bruising is common. High risk factors associated with vascular EDS often result in a life expectancy of about 50 years [source: Steiner].

The other three forms of EDS are rare, having been reported in fewer than 100 cases worldwide. Arthrochalasis, which is marked by congenital hip dislocation, has been seen in only about 30 cases [source: Mayo Clinic]. Patients with this type of EDS also experience early onset arthritis, increased bruising, stretchy skin and atrophic scarring, which marks the deterioration of tissue [source: Ehlers-Danlos National Foundation]. People with dermatosparaxis, which has been reported in a dozen cases around the world, have severely fragile skin with a soft, doughy texture. They, too, are highly susceptible to bruising, though the disorder does not inhibit wound healing as it does in other forms [source: Ehlers-Danlos National Foundation].

The least common form of EDS is kyphoscoliosis, which has been reported in only a few cases worldwide [source: Mayo Clinic]. Weak muscle tone and delayed motor development, often resulting in loss of movement during a person's 20s or 30s, are common in this EDS type [source: Ehlers-Danlos National Foundation]. With kyphoscoliosis, the spine becomes increasingly curved as the disease progresses, and the eyes, marked by small corneas, are easily damaged and ruptured.

Next, you will learn what impact these symptoms have on patients diagnosed with EDS.


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