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Due to deficient anchoring of the skin layers, even very slight mechanical stress increases the tendency of the skin to blister. This mainly concerns the skin on the hands and feet, but also mucous membranes (e.g. in the mouth and eyes) and membranes of the digestive tract. These blisters can be filled with blister secretions or blood, depending on the depth of the affected skin layers. If the deeper layers are affected, the healing process of the resulting wounds often leads to scarring and deformities, which in turn can cause contractures (e.g. of the hands and feet).
EB simplex is usually a mild form. The healing of the blisters usually occurs without scarring and skin atrophy. Blister formation usually occurs more during childhood than in adulthood.
There are both mild as well as early lethal forms of junctional EB. Healing without scarring is usually observed, however skin atrophy often occurs.
Dystrophic EB can either take a mild or a more severe form. Healing without scars is seldom, because the deeper skin layers are affected by blistering. In severe cases, bad scarring can cause mutilation of the hands and muscle contractures.
The level of blistering in the skin cannot be determined externally. Therefore secondary symptoms are important for the diagnosis of EB.
