Ehlers-Danlos Syndrome

This syndrome belongs to a group of inherited disorders characterized by flexible joints and stretchy and fragile skin. Connective tissues, mainly; skin, joints and blood vessels are affected in this syndrome. Connective tissues are made up of complex mixture of proteins and other substances that gives strength and elasticity to the structures underlying the body. Vascular Ehlers-Danlos syndrome, is a more serious and severe form of disorder in which walls of blood vessels, intestines or uterus tends to rupture.

Symptoms

Symptoms include:

Excessive flexibility of the joints
Joint pain
Dislocation of joints
Patient feel exceptionally soft and velvety. Skin become overly stretchy
Skin become fragile and healing of scars become delayed

Causes

Main factor in the development of Ehlers-Danlos syndrome is the genetic manifestation. If a parent has this syndrome, it’s likely they will pass it to there children.

Complications

Joint dislocation
Early-onset of arthritis
Prominent scarring
Fatal rupture of blood vessels in case of vascular Ehlers-Danlos syndrome.

Diagnosis

Diagnosis of this condition depends upon detailed medical history and complete general physical examination of the patient. Other test include genetic testing of blood samples.

Treatment

Although there’s no permanent cure for this syndrome. However, certain treatment options are there to manage the symptoms and prevention of complications.

Medications for pain and blood pressure control are prescribed.
Physical therapy to strengthen muscles and stabilize joints
Surgical repair is sometimes require to treat repeated dislocated joints

Ehlers-Danlos Syndrome