The British journal of dermatology | 2019
Opposing effects of STAT3 targeting in epidermolysis bullosa.
Abstract
Dystrophic epidermolysis bullosa (DEB) is a skin blistering disease caused by mutations in the COL7A1 gene encoding the anchoring fibril-constituent collagen VII.1 Secondary to skin fragility, DEB manifests as chronic wounds and progressive soft tissue fibrosis. As a consequence of a chronically injured and stiffened dermal microenvironment people with severe DEB are prone to develop early-onset metastatic cutaneous squamous cell carcinomas (cSCCs).1,2 Dermal fibrosis in DEB is paradigmatic of injury- and inflammation-driven activation of fibrogenic processes2 (and references therein).