Rarities in rare: Illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series

Amber Vanhaecke, Dieneke Schonenberg-Meinema, Sofie de Schepper, Sandy C. Bergkamp, Maria Comasia Leone, Maritza A. Middelkamp-Hup, Amara Nassar-Sheikh Rashid, J. Merlijn van den Berg, Taco W. Kuijpers, Annamaria Iagnocco, Maurizio Cutolo, Vanessa Smith

Research output: Contribution to journalArticleAcademicpeer-review


Objective. 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. Conclusion. 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.

Original languageEnglish
Pages (from-to)S12-S18
Number of pages7
JournalClinical and experimental rheumatology
Issue number5
Publication statusPublished - 1 May 2022


  • EULAR Study Group on Microcirculation in Rheumatic Diseases
  • durometer
  • laser speckle contrast analysis
  • localised scleroderma
  • microcirculation
  • nailfold videocapillaroscopy
  • skin fibrosis
  • ultrasonography

Cite this