Full Papers
Rarities in rare: illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series
A. Vanhaecke1, D. Schonenberg-Meinema2, S. De Schepper3, S.C. Bergkamp4, M.C. Leone5, M.A. Middelkamp-Hup6, A. Nassar-Sheikh rashid7, J.M. Van Den Berg8, T.W. Kuijpers9, A. Iagnocco10, M. Cutolo11, V. Smith12
- Department of Internal Medicine, Ghent University, and Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
- Department of Paediatric Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Amsterdam University Medical Centre (AUMC), University of Amsterdam, The Netherlands.
- Department of Dermatology, Ghent University Hospital, Ghent, Belgium.
- Department of Paediatric Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Amsterdam University Medical Centre (AUMC), University of Amsterdam, The Netherlands.
- Rheumatology Unit, S. Maria Hospital, Terni, Italy.
- Department of Dermatology, Amsterdam University Medical Centres (AUMC), University of Amsterdam, The Netherlands.
- Department of Paediatric Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Amsterdam University Medical Centre (AUMC), University of Amsterdam, The Netherlands.
- Department of Paediatric Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Amsterdam University Medical Centre (AUMC), University of Amsterdam, The Netherlands.
- Department of Paediatric Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Amsterdam University Medical Centre (AUMC), University of Amsterdam, The Netherlands.
- Academic Rheumatology Centre, Department of Clinical and Biological Science, University of Turin, Italy.
- Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy.
- Department of Internal Medicine, Ghent University and Department of Rheumatology, Ghent University Hospital; and Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. vanessa.smith@ugent.be
CER15136
2022 Vol.40, N°5 ,Suppl.134
PI 0012, PF 0018
Full Papers
Free to view
(click on article PDF icon to read the article)
PMID: 35084326 [PubMed]
Received: 04/09/2021
Accepted : 03/12/2021
In Press: 20/01/2022
Published: 18/05/2022
Abstract
OBJECTIVES:
To assess the (structural and functional) characteristics of the microvascular and dermal status in juvenile localised scleroderma (jLoS), using novel non-invasive standardised research tools commonly used in adult systemic sclerosis (SSc).
METHODS:
Ten consecutive patients with a confirmed jLoS diagnosis were studied cross-sectionally in this two-centre case series. For each patient, the most prominent lesion (i.e., “target lesion”) was chosen for further examination of the centre, edge and contralateral unaffected site. High-frequency ultrasonography was used to determine dermal thickness, durometer for skin hardness, and laser speckle contrast analysis (LASCA) for a dynamical evaluation of the microcirculation. The structure of the microcirculation was evaluated at the nailfolds of the 2nd-5th finger bilaterally, using nailfold videocapillaroscopy (NVC).
RESULTS:
6 linear and 4 plaque subtype jLoS lesions were included. Dermal thickness was thinner at the centre of the “target lesions” vs. the edges (p<0.001) and control sites (p<0.001). Skin hardness was harder at the centre of the “target lesions” vs. the edges (p=0.012) and control sites (p=0.003). A higher perfusion was found in the centre of the “target lesion” (124.87±66.40 PU) vs. the edges (87.27±46.40 PU; p<0.001) and control sites (67.85±37.49; p<0.001). Of note, all patients had a “non-scleroderma” pattern on NVC.
CONCLUSIONS:
This case series suggests the supportive value of both microcirculatory and dermal assessments of skin lesions using novel non-invasive research tools, adopted from adult SSc, for (j)LoS.
