Ehlers-Danlos Syndrome Types

Ehlers-Danlos syndrome (EDS) is a genetic disorder that may be recessive or dominant depending on the type of EDS you have. That means if you have a dominant type and have children, those children have a 50% chance of inheriting the disease. If you have a recessive type, each child has a 25% chance of inheriting the disease and a 50% chance of being a carrier of that disease. It is for this reason that many EDS sufferers consult with a genetic counselor, someone who can assess the risks of genetic disorders to you and your children, prior to having children. This is the only way to prevent the disease.

Ehlers-Danlos syndrome is classified into six major types or groups. It was originally divided into 11 different types, but scientists reclassified them based on signs and symptoms in 1997. This reclassification gave us the current six major types; hypermobility type, classical type, vascular type, kyphoscoliosis type, arthrochalasia type and dermatosparaxis type. There is a final grouping, other types, that is a catch-all group for the extremely rare cases that have only been seen in a handful of patients, usually all in the same family. All of the types affect your joints and many also affect your skin.

The hypermobility type is mainly characterized by loose, unstable joints and chronic joint pain. The loose joints increase the sufferer's risk of dislocating or straining the joint. Specific exercises to strengthen the muscles surrounding the joints will decrease the risk of dislocation and/or straining. You may find relief from the joint pain with over the counter pain medications. Your skin will be affected, but to what degree is uncertain. Hypermobility EDS is the most common type of EDS, affecting one in 10,000 to 15,000 people.

Classical EDS is the second most common type of EDS. It affects one in 20,000 to 40,000 people. The main symptoms with this type of EDS are very elastic skin that feels like velvet, bruises easily and is slow to heal wounds. Some sufferers also experience noncancerous skin growths on major pressure points (knees and elbows), shins and forearms. The joints are affected with this type, but not to the same degree as hypermobility EDS. They are prone to dislocation and may affect the large motor skill development in infants.

Vascular EDS is the most serious type of EDS because it primarily affects the blood vessels, making them very fragile and prone to tearing. Luckily, it is also one of the rarer forms, affecting only one in 100,000 to 200,000 people. This form also has elastic skin and loose joints, mainly the small joints of the fingers and toes. Sufferers of this type of EDS may also have very thin skin, making their veins very prominent.

The final three types, kyphoscoliosis, arthrochalasia and dermatosparaxis, are very rare. Only about 60 cases of kyphoscoliosis EDS have been reported worldwide. The main symptoms of this type of EDS are curving of the spine (scoliosis), eye problems including fragility and progressive muscle weakness. Arthrochalasia EDS is also rare; only 30 or so cases have been reported worldwide. This EDS is characterized by very loose joints, including the hips, which may affect the development of large motor skills such as walking. Sufferers of this type also have elastic skin that bruises easily, an earlier onset of arthritis and an increased risk of bone fractures and loss. The dermatosparaxis type of EDS is the rarest of the three, only about 10 cases have been reported worldwide. The primary symptom of this type is extremely loose and sagging skin. The joints are also affected, however, and may delay large motor skill development in children.

The final grouping, Others, is a catch-all grouping for the extremely rare and not well documented cases of EDS. The types here have a variety of symptoms, ranging from fragile skin without bruising or loose joints to chronic diarrhea and low blood pressure. If you suspect you have any of the types of EDS, you need to see your doctor right away.