Research in practice: Towards deciphering the role of epidermal proteases in recessive dystrophic epidermolysis bullosa progression.

Abstract

Recessive dystrophic epidermolysis bullosa (RDEB) is an incurable severe skin disease caused by loss of collagenVII, an extracellular protein that ensures skin cohesion. It manifests in skin blistering and unresolved cycles of wounding and healing that progressively lead to dermal stiffening and early development of aggressive cutaneous squamous cell carcinomas. Inflammation and subsequent tissue fibrosis highly contribute to RDEB pathogenicity and targeting them could provide new therapeutic options. Kallikreins (KLKs) are epidermal secreted proteases, which contribute to skin desquamation and inflammation. Kallikreins are involved in the pathogenesis of several inflammatory skin disorders, but interestingly also in the initiation and progression of different cancers. Our project aims at deciphering the role of KLKs in inflammation, fibrosis, and tumor development in RDEB.