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A systematic literature review of the disease burden in patients with recessive dystrophic epidermolysis bullosa.


ABSTRACT:

Background/objective

Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic collagen disorder characterized by skin fragility leading to blistering, wounds, and scarring. There are currently no approved curative therapies. The objective of this manuscript is to provide a comprehensive literature review of the disease burden caused by RDEB.

Methods

A systematic literature review was conducted in MEDLINE and Embase in accordance with PRISMA guidelines. Observational and interventional studies on the economic, clinical, or humanistic burden of RDEB were included.

Results

Sixty-five studies were included in the review. Patients had considerable wound burden, with 60% reporting wounds covering more than 30% of their body. Increases in pain and itch were seen with l

SUBMITTER: Tang JY 

PROVIDER: S-EPMC8045359 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature