Standard

Rarities in rare : illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series. / Vanhaecke, Amber; Schonenberg-Meinema, Dieneke; de Schepper, Sofie et al.

In: Clinical and experimental rheumatology, Vol. 40, No. 5, 01.05.2022, p. S12-S18.

Research output: Contribution to journalArticleAcademicpeer-review

Harvard

APA

Vancouver

Vanhaecke A, Schonenberg-Meinema D, de Schepper S, Bergkamp SC, Leone MC, Middelkamp-Hup MA et al. Rarities in rare: illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series. Clinical and experimental rheumatology. 2022 May 1;40(5):S12-S18. doi: 10.55563/clinexprheumatol/2vm1pz

Author

Vanhaecke, Amber ; Schonenberg-Meinema, Dieneke ; de Schepper, Sofie et al. / Rarities in rare : illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series. In: Clinical and experimental rheumatology. 2022 ; Vol. 40, No. 5. pp. S12-S18.

BibTeX

@article{8c803457210a4194bbb29a13017dde3a,
title = "Rarities in rare: illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series",
abstract = "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. ",
keywords = "ERN ReCONNET, EULAR Study Group on Microcirculation in Rheumatic Diseases, durometer, laser speckle contrast analysis, localised scleroderma, microcirculation, nailfold videocapillaroscopy, skin fibrosis, ultrasonography",
author = "Amber Vanhaecke and Dieneke Schonenberg-Meinema and {de Schepper}, Sofie and Bergkamp, {Sandy C.} and Leone, {Maria C.} and Middelkamp-Hup, {Maritza A.} and {Nassar-Sheikh Rashid}, Amara and {van den Berg}, {J. Merlijn} and Kuijpers, {Taco W.} and Annamaria Iagnocco and Maurizio Cutolo and Vanessa Smith",
note = "Funding Information: V. Smith is a Senior Clinical Investigator of the Research Foundation - Flanders (Belgium) (FWO) [1.8.029.20N]. The FWO was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. V. Smith is supported by an unrestricted educational chair on systemic sclerosis of Janssen-Cilag NV. Janssen-Cilag NV was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. Funding Information: V. Smith is a Senior Clinical Investigator of the Research Foundation – Flanders (Belgium) (FWO) [1.8.029.20N]. The FWO was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. V. Smith is supported by an unrestricted educational chair on systemic sclerosis of Janssen-Cilag NV. Janssen-Cilag NV was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. Publisher Copyright: {\textcopyright} Copyright Clinical and Experimental Rheumatology 2022.",
year = "2022",
month = may,
day = "1",
doi = "10.55563/clinexprheumatol/2vm1pz",
language = "English",
volume = "40",
pages = "S12--S18",
journal = "Clinical and experimental rheumatology",
issn = "0392-856X",
publisher = "Clinical and Experimental Rheumatology S.A.S.",
number = "5",

}

RIS

TY - JOUR

T1 - Rarities in rare

T2 - illuminating the microvascular and dermal status in juvenile localised scleroderma. A case series

AU - Vanhaecke, Amber

AU - Schonenberg-Meinema, Dieneke

AU - de Schepper, Sofie

AU - Bergkamp, Sandy C.

AU - Leone, Maria C.

AU - Middelkamp-Hup, Maritza A.

AU - Nassar-Sheikh Rashid, Amara

AU - van den Berg, J. Merlijn

AU - Kuijpers, Taco W.

AU - Iagnocco, Annamaria

AU - Cutolo, Maurizio

AU - Smith, Vanessa

N1 - Funding Information: V. Smith is a Senior Clinical Investigator of the Research Foundation - Flanders (Belgium) (FWO) [1.8.029.20N]. The FWO was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. V. Smith is supported by an unrestricted educational chair on systemic sclerosis of Janssen-Cilag NV. Janssen-Cilag NV was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. Funding Information: V. Smith is a Senior Clinical Investigator of the Research Foundation – Flanders (Belgium) (FWO) [1.8.029.20N]. The FWO was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. V. Smith is supported by an unrestricted educational chair on systemic sclerosis of Janssen-Cilag NV. Janssen-Cilag NV was not involved in study design, collection, analysis and interpretation of data, writing of the report, nor in the decision to submit the manuscript for publication. Publisher Copyright: © Copyright Clinical and Experimental Rheumatology 2022.

PY - 2022/5/1

Y1 - 2022/5/1

N2 - 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.

AB - 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.

KW - ERN ReCONNET

KW - EULAR Study Group on Microcirculation in Rheumatic Diseases

KW - durometer

KW - laser speckle contrast analysis

KW - localised scleroderma

KW - microcirculation

KW - nailfold videocapillaroscopy

KW - skin fibrosis

KW - ultrasonography

UR - http://www.scopus.com/inward/record.url?scp=85130766533&partnerID=8YFLogxK

U2 - 10.55563/clinexprheumatol/2vm1pz

DO - 10.55563/clinexprheumatol/2vm1pz

M3 - Article

C2 - 35084326

VL - 40

SP - S12-S18

JO - Clinical and experimental rheumatology

JF - Clinical and experimental rheumatology

SN - 0392-856X

IS - 5

ER -

ID: 24073918