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Different Forms of Epidermolysis Bullosa
Although there is a non inherited form of EB that can be acquired in adolescence or adulthood, this text focuses on
inherited forms of EB, which range from mild to severe and can require major adjustments in the lifestyle of both the
EB patient and his or her family.
Each type of EB presents somewhat different symptoms and affects the patient's day-to-day living in different ways. In
some instances, the right adjustments can allow the child to have a lifestyle that in many ways will not differ
dramatically from that of unaffected children. In other cases, the disorder is more complex and the child's life can be
seriously altered. The characteristics of the major inherited forms of EB will be described in the sections that follow.
There are three main forms of EB; EB Simplex, Junctional EB and Dystrophic EB. These types have various subtypes
as well. All types of EB vary in degrees of severity.
Skin is the body’s first line of defense. Its intricate matrix is composed of a top layer called the epidermis and a lower
layer called the dermis. Between the two layers is the basement membrane zone. Among the various proteins present
within the basement membrane zone are two layers, an upper lamina lucida and a lower layer, lamina densa.
In order to appropriately diagnose and classify the type of EB the person is affected with, a biopsy must be taken. The
biopsy procedure includes numbing an area and taking a small sample of skin for examination under a microscope.
The diagnostic testing for EB allows for the visualization of the framework of the sampled skin and determines at what
level the skin is separating or at what level blister formation occurs.
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