Extracutaneous features and complications of the Ehlers-Danlos syndromes: A systematic review.

ABSTRACT:

Introduction

The Ehlers-Danlos syndromes (EDS) comprise a group of inherited connective tissue disorders presenting with variable fragility to skin, soft tissue, and certain internal organs, which can cause significant complications, particularly arterial rupture, bowel perforation and joint difficulties. Currently, there are 14 proposed subtypes of EDS, with all except one subtype (hypermobile EDS) having an identified genetic etiology. An understanding of the extracutaneous features and complications within each subtype is key to maximizing clinical care and reducing the risk of further complications.

Methods

A systematic review of EDS-related extracutaneous features and complications was undertaken.

Results

We identified 839 EDS cases that met the inclusion criteri

SUBMITTER: Doolan BJ 

PROVIDER: S-EPMC9899794 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature