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Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies

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 hi...

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Autores principales: Hägerling, René, Van Zanten, Malou, Behncke, Rose Yinghan, Ulferts, Sascha, Hansmeier, Nils R., Märkl, Bruno, Witzel, Christian, Ho, Bernard, Keeley, Vaughan, Riches, Katie, Mansour, Sahar, Gordon, Kristiana, Ostergaard, Pia, Mortimer, Peter S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Clinical Investigation 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10619487/
https://www.ncbi.nlm.nih.gov/pubmed/37698920
http://dx.doi.org/10.1172/jci.insight.172179
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author Hägerling, René
Van Zanten, Malou
Behncke, Rose Yinghan
Ulferts, Sascha
Hansmeier, Nils R.
Märkl, Bruno
Witzel, Christian
Ho, Bernard
Keeley, Vaughan
Riches, Katie
Mansour, Sahar
Gordon, Kristiana
Ostergaard, Pia
Mortimer, Peter S.
author_facet Hägerling, René
Van Zanten, Malou
Behncke, Rose Yinghan
Ulferts, Sascha
Hansmeier, Nils R.
Märkl, Bruno
Witzel, Christian
Ho, Bernard
Keeley, Vaughan
Riches, Katie
Mansour, Sahar
Gordon, Kristiana
Ostergaard, Pia
Mortimer, Peter S.
author_sort Hägerling, René
collection PubMed
description 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 birthmarks and another 3 lesions as erythematous telangiectasias. The aims were (i) to demonstrate 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 color. Microscopy revealed all lesions as lymphatic structures. Port-wine birthmarks proved to be cystic lesions, with nonuniform lymphatic marker expression and a disconnected lymphatic network suggesting a lymphatic malformation. Erythematous telangiectasias represented expanded but nonmalformed lymphatics. Blood within lymphatics appeared to explain the color. 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 color accessing lymphatics through lympho-venous shunts.
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spelling pubmed-106194872023-11-02 Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies Hägerling, René Van Zanten, Malou Behncke, Rose Yinghan Ulferts, Sascha Hansmeier, Nils R. Märkl, Bruno Witzel, Christian Ho, Bernard Keeley, Vaughan Riches, Katie Mansour, Sahar Gordon, Kristiana Ostergaard, Pia Mortimer, Peter S. JCI Insight Research Article 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 birthmarks and another 3 lesions as erythematous telangiectasias. The aims were (i) to demonstrate 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 color. Microscopy revealed all lesions as lymphatic structures. Port-wine birthmarks proved to be cystic lesions, with nonuniform lymphatic marker expression and a disconnected lymphatic network suggesting a lymphatic malformation. Erythematous telangiectasias represented expanded but nonmalformed lymphatics. Blood within lymphatics appeared to explain the color. 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 color accessing lymphatics through lympho-venous shunts. American Society for Clinical Investigation 2023-10-23 /pmc/articles/PMC10619487/ /pubmed/37698920 http://dx.doi.org/10.1172/jci.insight.172179 Text en © 2023 Hägerling et al. https://creativecommons.org/licenses/by/4.0/This work is licensed under the Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Research Article
Hägerling, René
Van Zanten, Malou
Behncke, Rose Yinghan
Ulferts, Sascha
Hansmeier, Nils R.
Märkl, Bruno
Witzel, Christian
Ho, Bernard
Keeley, Vaughan
Riches, Katie
Mansour, Sahar
Gordon, Kristiana
Ostergaard, Pia
Mortimer, Peter S.
Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
title Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
title_full Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
title_fullStr Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
title_full_unstemmed Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
title_short Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
title_sort erythematous capillary-lymphatic malformations mimicking blood vascular anomalies
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10619487/
https://www.ncbi.nlm.nih.gov/pubmed/37698920
http://dx.doi.org/10.1172/jci.insight.172179
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