Epidermolysis Bullosa (EB for short) is an umbrella term for a group of disorders characterized by blistering of the body. EB is an inherited disease that is present from birth and can not be cured.
The predisposition for the disease by one or both parents passed on. Since it depends on the type of EB or the construction inherits recessive or dominant, it may be that a newborn child with EB is the first in his family with the disease (only in case of recessive inheritance). Recessive inheritance means that both parents are carrying construction for EB, but themselves are healthy. As a child of either parent gets the piece of construction, the child or EB.
The construction that is transferred is an error in one of the genes in the body responsible for the production of proteins. These proteins normally provide stability and a good adhesion between cells in the epidermis and a good adhesion between the epidermis and dermis. When a person with EB is not enough of the normal protein is produced or there is an abnormal protein produced. The adhesion between and within the skin layer is disrupted and the skin layers do not fit easily and blistering. The blistering already slight friction and even simple actions like holding a pen (on the hands and fingers) or a walk (on foot). The blisters can occur over the body, depending on the type of EB.
So typical of EB blisters, which can lead to open sores on the skin and in severe forms also deformities of toes and fingers by the formation of scar tissue.
Different forms of EB
EB can be divided into three groups, each of which have a number of subtypes. The distribution is determined by the location of the protein where the error is. This is also where the blistering.
EB symplex: In this form of EB caused the blisters in the epidermis. The error in the protein, the strength of the affected cells in the epidermis.
Junctional EB: birthplace of blisters in the lower cells of the epidermis. The epidermis release of the membrane into the dermis, which blisters form.
Dystrophic EB: The blistering just below the membrane in the dermis.
The typographical error in the given protein or blisters on only certain parts of the body prevent or over the body.
Treatment of EB
The treatment of EB is to provide the blisters. The wound care is time consuming and painful, because the blisters after treatment often quickly return. Treatment aims to sweeten the daily life of the patient. The disease is (still) incurable and can be deadly serious. The biggest danger to the disease EB is the constant risk of infection.