Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues primarily your skin, joints and blood vessel walls. Connective tissue is a complex mixture of proteins and other substances that provides strength and elasticity to the underlying structures in your body. A more severe form of the disorder, called vascular Ehlers-Danlos syndrome, can cause the walls of your blood vessels, intestines or uterus to rupture. Different subtypes of Ehlers-Danlos syndrome are associated with a variety of genetic causes, some of which are inherited and passed on from parent to child.
Signs and symptoms of the most common form of Ehlers-Danlos syndrome include: Overly flexible joints (Because the connective tissue that holds joints together is looser, your joints can move far past the normal range of motion), Stretchy skin (Weakened connective tissue allows your skin to stretch much more than usual.), Fragile skin (Damaged skin often doesn't heal well.)
There is no particular cure for Ehlers-Danlos Syndrome. But few treatments can help to manage symptoms. Use of pain relievers, Anti-hypertensive drugs and surgical procedures are the treatments. The prevalence of Ehlers-Danlos syndrome is reported to be 1 case in approximately 400,000 people, but mild or incomplete forms appear to be underdiagnosed and more common than other forms. Type IV Ehlers-Danlos syndrome is a severe form. Patients often have a shortened lifespan because of the spontaneous rupture of a large artery (eg, splenic artery, aorta) or the perforation of internal organs. Surgery can pose life-threatening risks in these patients. No racial predominance seems to exist; however, some believe that whites probably are affected more than other races. The disease has clinical features (eg, joint mobility, skin extendibility, scarring tendency) that are easily recognizable beginning in early childhood.