Safety and Efficacy of Angiotensin Receptor Antagonists in Recessive Dystrophic Epidermolysis Bullosa.

Abstract

Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited progressive blistering skin disorder caused by mutations in the COL7A1 gene. Chronic wounds lead to cycles of scarring and healing, causing severe functional deformities. Losartan is an angiotensin II type 1 receptor (AT1R) antagonist that has been shown to have antifibrotic activity via inhibiting the production of transforming growth factor beta (TGF-B) and has a favourable safety profile in children. In this systematic review, there were five studies found, with a total of 59 patients including case series, case-control and an open label phase 2 clinical trial. These studies have demonstrated the safety of losartan with no significant adverse effects such as hypotension, hyperkalaemia and hypersensitivity. They have also demonstrated evidence of efficacy utilising subjective assessments and objective criteria such as the Birmingham Epidermolysis Bullosa Severity score (BEBS) and Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI). Although safe and effective, further clinical trials are required to fully elucidate the role of this treatment; whether this approach should be standardised across the RDEB patient cohort is yet to be determined from the current evidence. Trial Registration: PROSPERO ID: CRD42024557126.