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Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies.

Hägerling, R; Van Zanten, M; Behncke, RY; Ulferts, S; Hansmeier, NR; Märkl, B; Witzel, C; Ho, B; Keeley, V; Riches, K; et al. Hägerling, R; Van Zanten, M; Behncke, RY; Ulferts, S; Hansmeier, NR; Märkl, B; Witzel, C; Ho, B; Keeley, V; Riches, K; Mansour, S; Gordon, K; Ostergaard, P; Mortimer, PS (2023) Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies. JCI Insight, 8 (20). e172179. ISSN 2379-3708 https://doi.org/10.1172/jci.insight.172179
SGUL Authors: Ostergaard, Pia Mansour, Sahar

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Abstract

Superficial erythematous cutaneous vascular malformations are assumed to be blood vascular in origin, but cutaneous lymphatic malformations can contain blood and appear red. Management may be different and so an accurate diagnosis is important. Cutaneous malformations were investigated through 2D-histology and 3D-whole-mount-histology. Two lesions were clinically considered as port-wine birthmark, and another three lesions as erythematous telangiectasias.The aims were: i) to prove that cutaneous erythematous malformations including telangiectasia can represent a lymphatic phenotype, ii) to determine if lesions represent expanded but otherwise normal or malformed lymphatics, and iii) to determine if the presence of erythrocytes explained the red colour. Microscopy revealed all lesions as lymphatic structures. Port-wine birthmarks proved to be cystic lesions, with non-uniform lymphatic marker expression, and a disconnected lymphatic network suggesting a lymphatic malformation. Erythematous telangiectasias represented expanded but non-malformed lymphatics. Blood within lymphatics appeared to explain the colour. Blood-lymphatic-shunts could be detected in the erythematous telangiectasia.In conclusion, erythematous cutaneous capillary lesions may be lymphatic in origin but clinically indistinguishable from blood vascular malformations. Biopsy is advised for correct phenotyping and management. Erythrocytes are the likely explanation for colour accessing lymphatics through lympho-venous-shunts.

Item Type: Article
Additional Information: Copyright: © 2023, Hägerling et al. This is an open access article published under the terms of the Creative Commons Attribution 4.0 International License.
Keywords: Angiogenesis, Cell Biology, Diagnostic imaging, Lymph
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: JCI Insight
ISSN: 2379-3708
Language: eng
Dates:
DateEvent
23 October 2023Published
12 September 2023Published Online
6 September 2023Accepted
Publisher License: Creative Commons: Attribution 4.0
Projects:
Project IDFunderFunder ID
UNSPECIFIEDBerlin Institute of Healthhttp://dx.doi.org/10.13039/501100017268
UNSPECIFIEDLymphatic Malformation InstituteUNSPECIFIED
MR/P011543/1Medical Research Councilhttp://dx.doi.org/10.13039/501100000265
RG/17/7/33217British Heart Foundationhttp://dx.doi.org/10.13039/501100000274
UNSPECIFIEDCharité – Universitätsmedizin Berlinhttp://dx.doi.org/10.13039/501100002839
101078827European Research Councilhttp://dx.doi.org/10.13039/501100000781
PubMed ID: 37698920
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/115701
Publisher's version: https://doi.org/10.1172/jci.insight.172179

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