Saturday, May 4, 2013

How many types of Ehlers-Danlos syndrome are there and why I feel so odd and alone among people I know.

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.

In the past, there were more than 10 recognized types of Ehlers-Danlos syndrome. In 1997, researchers proposed a simpler classification that reduced the number of major types to six and gave them descriptive names: the arthrochalasia type, the classic type, the dermatosparaxis type, the hypermobility type, the kyphoscoliosis type, and the vascular type. Other forms of the condition may exist, but they have been reported only in single families or are not well characterized.

Although all types of Ehlers-Danlos syndrome affect the joints and may also affect the skin, features vary by type. An unusually large range of joint movement (hypermobility) occurs with most forms of Ehlers-Danlos syndrome, particularly the hypermobility type. Infants with hypermobile joints often appear to have weak muscle tone, which can delay the development of motor skills such as sitting, standing, and walking. The loose joints are unstable and prone to dislocation, chronic pain, and early-onset arthritis. Dislocations involving both hips are a characteristic finding in infants with the arthrochalasia type of Ehlers-Danlos syndrome.

Many people with Ehlers-Danlos syndrome have soft, velvety skin that is highly elastic (stretchy) and fragile. Affected individuals tend to bruise easily, and some types of the condition also cause abnormal scarring. People with the classic form of Ehlers-Danlos syndrome experience wounds that split open with little bleeding and leave scars that widen over time to create characteristic shallow "cigarette paper" scars. The dermatosparaxis type of the disorder is characterized by skin that sags and wrinkles. Extra (redundant) folds of skin may be present as affected children get older.

Some forms of Ehlers-Danlos syndrome, notably the vascular and kyphoscoliosis types, can involve serious and potentially life-threatening complications. Blood vessels can tear (rupture) unpredictably, causing internal bleeding, stroke, and shock. The vascular type of Ehlers-Danlos syndrome is also associated with an increased risk of organ rupture, including tearing of the intestine and rupture of the uterus (womb) during pregnancy. People with the kyphoscoliosis form of Ehlers-Danlos syndrome experience severe, progressive curvature of the spine that can interfere with breathing.

I have Classical Type I Ehlers-Danlos. The classic type probably occurs in 1 in 20,000 to 40,000 people. This number made me wonder what that would look like so I thought about Cowboys Stadium in Dallas which holds around 80,000 people which means that on any given Sunday when the Cowboys are playing there myself and maybe one other person in the whole crowd would have the type of Ehlers-Danlos that I have. That put it in perspective for me just how rare this is and why most doctors are not familiar with it. My primary care doctor is in his 60s so he's been practicing for a long time and he told me that I'm the only patient that he's ever seen with Ehlers-Danlos. I can't imagine how many patients he's seen in his career which again puts it in perspective for me of how rare this is.

The lighting at the beginning of the video is not good at all but keep watching because it gets better.
This video shows a few of the basic things about Ehlers-Danlos Syndrome Classical Type I. This information is based on my own experience. One of the major complications I have with Ehlers-Danlos and another condition I have which is like a double whammy is concentration problems so please forgive my rambling and searching for the correct word in the video. Another problem with Ehlers-Danlos syndrome is chronic fatigue so please pardon the fact that I look like I'm half asleep.

This chart shows some of the things that an EDS body can do that is out of the range of normal flexibility. 


  1. thank you for the information. I am 33 and I think I have this. my skin is relly soft and streachy and I have the nose and ears that are bendy. Also my fingers can touch my arm backwards. Have had symptoms since my youth and never knew what it was. my arthritis is now debilitating. hope to find a dr to diagnose me and help me manage the constant pain.


  2. Camilla I am so glad that you found this info helpful. A great resource for more info on EDS is at If you have an orthopedic dr he/she should be able to help with diagnosing you. You may even want to print out the chart above, don't know if you can print it from here but google EDS images and you will find it or one similar, and take it to your PC and let he/she help you find a dr. to see.

    I hope you get the help you need!!!
    Gentle hugs!