Article
Pathomechanisms of Altered Wound Healing in Recessive Dystrophic Epidermolysis Bullosa.
The American journal of pathology - 1 Jul 2017
Cianfarani Francesca, Zambruno Giovanna, Castiglia Daniele, Odorisio Teresa
Abstract excerpt
Individuals with recessive dystrophic epidermolysis bullosa (RDEB), a rare genetic skin disease, carry mutations in the COL7A1 gene that codes for type VII collagen, an extracellular matrix component of the basement membrane zone forming the anchoring fibrils. As a consequence, RDEB individuals manifest unremitting skin blistering that evolves into chronic wounds, inflammation, and fibrosis. These features play a...
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