Human skin suffers from some prevalent ailments some of which are inherited while the others are caused by external factors. Epidermolysis bullosa is a rare and significant skin disorder that causes the afflicted person’s skin to become abnormally fragile and develops recurring blisters on the surface.
It makes the skin extremely sensitive to touch and external matters. It is a genetic ailment that should be thought of as a group of conditions that lead to the vulnerability to skin to surrounding elements.
The human skin can be divided into two main layers. The inner layer is called dermis while the outer layer is known as epidermis. The people born with normal skin structure have anchors between these 2 layers of skin. The anchors stop the layers from stretching and moving on their own. People who inherit Epidermolysis bullosa do not have these anchors that may hold them together.
As a result, if something makes friction between dermis and epidermis, he gets painful sores and blisters. The younger people born with such skin are described as "Butterfly Children" since the fragility of their skin is compared to that of the wings of butterfly.
This skin disorder can be seen in 9 out of every 1 million people. As a matter of fact, this genetic disease can affect both women and men and people belonging to any race can be affected.
Symptoms of Epidermolysis Bullosa
The symptoms of the disease depend on which variant the patient is afflicted with. The symptoms are also subject to the skin layer that is affected. However, the most prevalent symptom is blistering. The implications of these may be moderate to severe.
The most common variant of the ailment is EB simplex. This affects the epidermis layer of the victim. In this type, the blisters appear only in the victim’s feet and hand. In some instances, they can appear all over the body. This type of Epidermolysis bullosa may become visible in kids and also in grown up people.
The variant called Junctional Epidermolysis bullosa may impact only about 10% of the people suffering from the disease. It is comparatively more acute in nature. Children who are affected with this variant do not live long.
People affected with Dystrophic Epidermolysis bullosa may become disabled as their body parts are severely scarred including feet, hands and face.
Causes And Types Of Epidermolysis Bullosa
A person who contracts this disease lack the chemicals that plays a vital role in keeping the layers of skin as one. It is inherited by the victims. There are around 27 variants of the disease that have been discovered so far, but its 3 main variants are dystrophic EB, junctional EB and EB simplex. The various sub categories are characterized by the blister location in the layers of the skin.
The EB simplex can be sub divided into the following 4 categories- EBS-WC, EBS-DM, EBS-MP and EBS-K.
The second variant of the disease, Junctional EB, is marked by unprompted blistering of the person’s skin. In it, the mucous membranes are also affected severely. The defective structure of laminin 6, laminin 5 as well as collagen XVII and some proteins responsible for the unity of skin layers lead to the development of this disorder. Patients suffering from this ailment embrace death early since the digestive system, the respiratory and the genitourinary systems of his body are severely affected by intermittent blistering.
The Dystrophic EB causes scarring and abrasions in deeper tissue level within the upper part of dermis.
Treatment for Epidermolysis Bullosa
This genetic disorder has no specific remedy. The available treatments include reducing injury and friction. The infections caused by the blisters also need to be treated to mitigate the chances of chronic damage. There is the provision of conducting antenatal tests for Epidermolysis bullosa. It is generally performed after 8 to 10 weeks of pregnancy.
