The Ehlers-Danlos Syndrome comes in a group of several disorders of the connective tissue. Hereditary, the disorders are caused by various different defects in collagen synthesis. However, the latest disorder to join the group, although hereditary, does not claim defects in synthesis of collagen as its cause. Instead, abnormalities are usually found in the tenascin protein which is thought to be influential in the binding together of cells in tissues. The classification of types of EDS is based on their signs and symptoms.
If your skin is velvety, smooth, stretches unusually and gets bruised easily, you should be checked for this type of Ehlers-Danlos Syndrome. Other prominent characteristics of Classical EDs include the tendency to develop wounds without the usual amount of accompanying blood but ones that take an abnormally long period to heal. In addition, your joints may be loose and cover an extraordinary long range of movement, a condition known as hypermobility.
In infants, hypermobility as a result of the syndrome may be characterised by weak muscle tone. Consequently, the infant's abilities in such motor skills as standing, walking and even sitting are affected, resulting to delayed growth and development. The patient may also suffer from mitral valve prolapse, a condition that affects proper flow of blood between the heart chambers.
It was formerly known as EDS type III. It is the most common type of Ehlers-Danlos Syndrome since it is more likely to be inherited than all other types. As the name suggests, the patient's joints have an abnormal range of movement and together with limbs, begin to chronically pain early in the person's life. In most cases, there is instability in both the small and large joints while there is easy and frequent dislocation of other joints including knee, shoulder and jaw.
When a person has this type of Ehlers-Danlos Syndrome, even a minor trauma may result to rupturing of blood vessels or organs. It is, therefore, regarded as the most serious type of EDS. Additionally, its diagnosis is very difficult and there are many cases of misdiagnosis.
Some of its major symptoms include thin and delicate skin that is prone to bruises. Moreover, the veins may show through the skin on the hands, feet, chest and abdomen. Nevertheless, the skin is velvety without having be to stretchy as evident with other types of EDS.
Patients with Vascular EDS are at a high risk of death due to complications arising from ruptured arteries and organs. They include internal bleeding, shock or stroke. Therefore, x-rays should be replaced by MRI, MRA or CT-Scan in order to ensure diagnosis is conducted in the most appropriate way for timely treatment.
When an infant is born with weak muscle tone and laxity of the joints as well as scoliosis, this type of Ehlers-Danlos Syndrome is probably the cause. After he or she is 20 or 30 years old, there may be loss of ambulation. In addition, a minor injury may result to the rupturing of the ocular globe.
As in most of the other types of EDS, the patient's skin may bruise easily. Moreover, there is presenceof atrophic scars. There is also a delay in motor skills development in infants.
In most cases, people with this type of Ehlers-Danlos Syndrome experience congenital dislocation of the hip. In addition, they exhibit signs of severe joint hypermobility accompanied by regular part-dislocations also known as subluxations. In addition, there may be presence of Kyphoscoliosis, which is a combination of scoliosis and kyphosis - lateralcurvature and outward curvature of the spine respectively. The general signs of EDS will also be sure to show up. They include fragility of skin tissues with atrophic scars and muscle hypotonia.
While patients of this type of Ehlers-Danlos Syndrome exhibit most of the other signs of that come with the other types including fragility of the skin and bruising, the wounds heal as quickly as any other and the scars are not atrophic. However, the skin sags and it has a soft texture. The patients of Dermatosparaxis EDS are few.
This type of Ehlers-Danlos Syndrome is the latest to join the group. Its main symptoms include fragile and stretchy skin as well as hypermobility. However, the scars in the skin are not atrophic. This type comes up in only one generation of members of a family.
Loose joints and fragile skin and tissue fragility are the most common signs in all types of Ehlers-Danlos Syndrome. They usually come about as a result of production of abnormal proteins by abnormal genes leading to inheritance of frail collagen (with the exception of Tenascin-X Deficient EDS). Apart from several mutations, every type of EDS is unique to a particular family. Therefore, you're not likely to find an offspring with one type of EDS while the parents have another.