Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies

René Hägerling; Malou Van Zanten; Rose Yinghan Behncke; Sascha Ulferts; Nils R. Hansmeier; Bruno Märkl; Christian Witzel; Bernard Ho; Vaughan Keeley; Katie Riches; Sahar Mansour; Kristiana Gordon; Pia Ostergaard; Peter S. Mortimer

doi:10.1172/jci.insight.172179

Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies

René Hägerling,¹ Malou Van Zanten,² Rose Yinghan Behncke,¹ Sascha Ulferts,¹ Nils R. Hansmeier,¹ Bruno Märkl,³ Christian Witzel,⁴ Bernard Ho,⁵ Vaughan Keeley,⁶ Katie Riches,⁶ Sahar Mansour,⁷ Kristiana Gordon,⁵ Pia Ostergaard,² and Peter S. Mortimer²

Published September 12, 2023 - More info

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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.

Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies

René Hägerling,¹ Malou Van Zanten,² Rose Yinghan Behncke,¹ Sascha Ulferts,¹ Nils R. Hansmeier,¹ Bruno Märkl,³ Christian Witzel,⁴ Bernard Ho,⁵ Vaughan Keeley,⁶ Katie Riches,⁶ Sahar Mansour,⁷ Kristiana Gordon,⁵ Pia Ostergaard,² and Peter S. Mortimer²

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Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies

René Hägerling,1 Malou Van Zanten,2 Rose Yinghan Behncke,1 Sascha Ulferts,1 Nils R. Hansmeier,1 Bruno Märkl,3 Christian Witzel,4 Bernard Ho,5 Vaughan Keeley,6 Katie Riches,6 Sahar Mansour,7 Kristiana Gordon,5 Pia Ostergaard,2 and Peter S. Mortimer2

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René Hägerling,¹ Malou Van Zanten,² Rose Yinghan Behncke,¹ Sascha Ulferts,¹ Nils R. Hansmeier,¹ Bruno Märkl,³ Christian Witzel,⁴ Bernard Ho,⁵ Vaughan Keeley,⁶ Katie Riches,⁶ Sahar Mansour,⁷ Kristiana Gordon,⁵ Pia Ostergaard,² and Peter S. Mortimer²