Dystrophic Epidermolysis bullosa (DEB)
Dystrophic Epidermolysis bullosa is an umbrella term for all forms of EB, where the blisters are formed in relation to the dermis layer of the skin.
„Dystrophic“ originates from the ancient Greek syllable „dys“ and means „bad“ and „tropein“ for „feed“ and „grow“. Since the people suffering with this form are struggling with difficult food problems more than others, this gave the name to these forms.
In DEB in addition to the blistering of the skin there are usually additional problems that we discuss in more detail with the different forms.
Dystrophic EB Subtypes
In all forms of DEB it is always the same block or always the same protein molecule of genetic changes involved, namely the collagen 7. These changes can then lead to the collagen 7 to be completely missing, or is greatly reduced or somewhat limited in its function. There are different degrees of severity of DEB depending on the changes in collagen 7. It is important to know the exact diagnosis when DEB is suspected, if you want to adjust to the further course of the disease.
Important points in a nutshell
- Dystrophic Epidermolysis bullosa (DEB) is an umbrella term for all forms of EB, where the blister formation occurs in the bottom layer of the skin, the dermis.
- DEB is caused by different changes to a particular gene, the gene for collagen 7.
- The modes of transmission are different, there are recessive and dominant inherited dystrophic forms of EB.
- The individual's forms differ significantly, depending on whether collagen 7 is completely absent, reduced and/or limited in its function.
- In addition to the blistering on the skin, there are often additional problems (involvement of the mucous membranes and changes to the hands among other things).