The cortex layer adheres to the dermis layer by means of a very thin, complex area that can only be seen with an electron microscope. It is called the basement membrane area. It connects the basal epidermal cells with the upper dermis fibers. The basement membrane has an important property in preventing the epidermis from separating from the dermis when the skin is subjected to friction, trauma, or minor injuries. The skin is exposed to it every minute during normal daily activity.
Bullous epidermolysis is characterized by the appearance of bubbles that end with blisters on the skin areas exposed to simple friction and in the event of any injury or bump – even if it is simple – as a result of congenital genetic defects in the basement membrane area.
A few days after the birth of the child, the parents notice the appearance of bubbles on the areas of friction such as the anklets of the feet, the knees, the hands, and the areas of the torso that are subject to friction, leaving areas of peeled skin that heal to reveal new bubbles when another friction occurs, and so on.
In severe cases, bubbles may appear on the oral mucosa, and skin healing may be followed by scars, adhesions between the fingers, and deformities.
Studies conducted on this rare disease have shown that there are many types that differ in their severity, how they are transmitted through inheritance, and the course of the disease.
Epidermolysis bullosa is a rare genetic disease, but it causes the patient and his family many problems, especially when the child begins to crawl, then walk, run and play. Unfortunately, the treatment of such cases is still limited to trying to protect the skin from friction and injury, which is also a difficult requirement to achieve.
