Abstract
Dystrophic epidermolysis bullosa (DEB) is an inherited skin disorder, characterized by mutations in the gene encoding for collagen VII. DEB manifests in chronic skin fragility with blistering at the dermal-epidermal junction. Unremitting injury and inflammation evoke progressive fibrosis of skin and other epithelia in DEB. Here, we aimed to understand consequences of this with respect to biomechanics, epidermal integrity and barrier function. To this end, we followed changes in skin biomechanics with age in DEB mice using atomic force microscopy and its consequences using lipidomics, followed by validating molecular analyses in human DEB donor skin.
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