Glomeruloid haemangioma in Erdheim–Chester disease: An atypical skin manifestation associated with elevated vascular endothelial growth factor‐A levels

Glomeruloid haemangiomas and extensive angiomas occurred in a small subset of Erdheim-Chester disease patients, all showing markedly elevated vascular endothelial growth factor-A (VEGF-A) levels despite the absence of POEMS (polyneuropathy, organomegaly, M-spike, and skin disease) syndrome. Molecular analyses demonstrated mitogen-activated protein kinase (MAPK)-activated histiocytes without endothelial mutations, supporting a paracrine VEGF-A-driven angiogenesis mechanism underlying these atypical cutaneous lesions.

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Publication Details

Journal
British Journal of Haematology
Published
2026-06-19
DOI
https://doi.org/10.1111/bjh.70627
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

Glomeruloid haemangioma in Erdheim–Chester disease: An atypical skin manifestation associated with elevated vascular endothelial growth factor‐A levels

Félix Blaison, Francesco Pegoraro, Jean‐François Emile, Paul Legendre et al.
British Journal of Haematology
Histiocytic Disorders and Treatments
article

Glomeruloid haemangioma in Erdheim–Chester disease: An atypical skin manifestation associated with elevated vascular endothelial growth factor‐A levels

Félix Blaison, Francesco Pegoraro, Jean‐François Emile, Paul Legendre, Matthias Papo, Julien Haroche, Valentin Carado, Augusto Vaglio, Martin Caretti, Aubriot Lorton Marie Helene, Jerome Razanamahery
article en

Abstract

Glomeruloid haemangiomas and extensive angiomas occurred in a small subset of Erdheim-Chester disease patients, all showing markedly elevated vascular endothelial growth factor-A (VEGF-A) levels despite the absence of POEMS (polyneuropathy, organomegaly, M-spike, and skin disease) syndrome. Molecular analyses demonstrated mitogen-activated protein kinase (MAPK)-activated histiocytes without endothelial mutations, supporting a paracrine VEGF-A-driven angiogenesis mechanism underlying these atypical cutaneous lesions.

British Journal of Haematology
Université de Bordeaux (FR), Sorbonne Université (FR), Bordeaux Population Health (FR), Pitié-Salpêtrière Hospital (FR), Centre Hospitalier du Mans (FR), Hôpital Ambroise-Paré (FR), Maison des Sciences sociales et des Humanités de Dijon (FR), University of Florence (IT)
Zero hunger
Openalex Percentile: Top 8%
Histiocytic Disorders and Treatments
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Glomeruloid haemangioma in Erdheim–Chester disease: An atypical skin manifestation associated with elevated vascular endothelial growth factor‐A levels — Félix Blaison, Francesco Pegoraro, et al. · British Journal of Haematology (2026) | TGRS Research Map | TGRS