Full Papers
Microvascular status and skin thickness in adults with hypermobile Ehlers-Danlos syndrome: a pilot investigation
A. Sulli1, E. Hysa2, A. Cere3, F. Lalli4, A. Pinelli5, S. Sammorì6, E. Gotelli7, C. Pizzorni8, F. Malfait9, M. Castori10, V. Smith11, M. Cutolo12
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy. albertosulli@unige.it
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
- Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.
- Division of Medical Genetics, Fondazione IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy.
- Department of Internal Medicine and Department of Rheumatology, Ghent University Hospital, Ghent; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre, Ghent, Belgium.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, and IRCCS Ospedale Policlinico San Martino, Genova, Italy.
CER16908
2024 Vol.42, N°3
PI 0682, PF 0688
Full Papers
PMID: 37976114 [PubMed]
Received: 13/06/2023
Accepted : 18/09/2023
In Press: 08/11/2023
Published: 27/03/2024
Abstract
OBJECTIVES:
Hypermobile Ehlers-Danlos Syndrome (hEDS) is a hereditary connective tissue disorder characterised by joint hypermobility, chronic musculoskeletal pain, and skin abnormalities and easy bruising. Morphological and functional microvascular status has not yet been studied in hEDS, and dermal thickness (DT) has been poorly investigated.
METHODS:
The aim of the study was to investigate the microvascular morphology by nailfold videocapillaroscopy (NVC), peripheral blood perfusion (PBP) by laser speckle contrast analysis (LASCA), and DT by high-frequency skin ultrasound (22 MHz probe) in adults with hEDS compared to sex- and age-matched controls.
RESULTS:
Microhaemorrhages were found more prevalent and the capillary number per linear millimetre at the nailfold was slightly higher in hEDS patients than in controls, as well as the NVC score for abnormal shaped capillaries was slightly lower (less abnormal shaped capillaries) in hEDS patients than in controls, even if this was not statistically significant. PBP was comparable between hEDS patients and controls. The DT resulted generally lower in hEDS patients than controls with significant values limited to feet and thorax (p=0.04). A statistically significant positive correlation was observed between the Beighton score and the score for microhaemorrhages (r=0.4, p=0.05), as well as between the Beighton score and DT (r≥0.5, p≤0.02) at the level of feet and thorax.
CONCLUSIONS:
Our study detected in hEDS patients a normal microvascular function at rest and a suitable capillary morphology but with increased microvascular fragility. The dermal thickness seems thinner in hEDS patients than in controls in most skin areas, with strong statistically significance at the level of feet and thorax.
