Ehlers–Danlos syndromes (EDS): Symptoms, complications

EDS is a group of disorder of the connective tissues affects the skin, bones, blood vessels and even few organs and tissues. The symptoms can either be mild or even life-threatening.

Many people with Ehlers–Danlos syndrome have soft velvety skin that is highly stretchy and fragile and overly flexible joints. In 2017, it was classified and described in 13 different types.

Symptoms:

Symptoms can be classified under classic Ehlers–Danlos syndrome and vascular type of Ehlers–Danlos syndrome.

• Overly flexible joints, the joints are highly flexible, since the connective tissue around the joints are loose causing joints to move more than the normal range of movement. Since the joints are overly flexible there is an increased chance of joints dislocated and pain.
• Stretchy skin, the connective tissues are weakened allowing the skin to stretch much more than normal.
• Chronic fatigue
• The skin is so flexible that it can be pinched up away from the flesh, but once it is left it will go back to its original place. The skin is extremely velvety and soft.
• Fragile skin, the damaged skin takes time to heal and does not heal properly. The wounds take time to heal leaving a thin crinkly scar behind.

Symptoms severity varies from person to person. Few people exhibit only symptoms of the bones of overly flexible joints without any expression of the skin symptoms.

Symptoms of Ehlers–Danlos syndrome, the vascular type

It is seen in fair skinned person with visible underlying blood vessels. The facial features are very prominent in people suffering from the vascular type of Ehlers–Danlos syndrome, with the small earlobe, thin nose, and thin upper lip. In the vascular type of the disease, the blood vessels of the body are weakened. If aorta or any other major arteries are weakened and ruptures, then that could be fatal.

The vascular type also weakens the walls of the intestine and uterus which is also at risk of being ruptured.

Causes:

• Genetic causes
• Family history

Complications:

• Joints dislocation
• Early onset of arthritis
• Fatal rupture of blood vessels
• Prominent scarring
• Risk during pregnancy
• Rupture of organs like the uterus and intestine.

Lifestyle modifications:

• Learn to use joints correctly with the help of a physiotherapist.
• Reduce stress levels.
• Exercise regularly as advised by the physiotherapist
• Quit smoking tobacco

When to see a doctor?

If you experience symptoms of Ehlers–Danlos syndrome, see a doctor. If you are already diagnosed with the same and you are expecting a baby visit a doctor to prevent complications during pregnancy.
In case of pregnancy do visit a generic counselor to reduce the chances of the foetus to inherit the disease.

 

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