Glomeruloid haemangioma: a possible consequence of elevated VEGF in POEMS and Erdheim-Chester disease

Background Glomeruloid haemangioma (GH) is considered a specific marker of POEMS syndrome, despite some published GH cases unrelated to POEMS syndrome. Objectives To present two cases with GH and atypical presentations of Erdheim-Chester disease (ECD) or POEMS syndrome, as well as a retrospective mo...

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Bibliographic Details
Published in:EJD. European journal of dermatology 2018-11, Vol.28 (6), p.784-789
Main Authors: Legendre, Paul, Norkowski, Emma, Le Pelletier, François, Miyara, Makoto, Choquet, Sylvain, Haroche, Julien, Brocheriou, Isabelle, Barete, Stéphane
Format: Article
Language:English
Subjects:
Adult
Aged
Aged, 80 and over
Dermatology
Erdheim-Chester Disease - blood
Erdheim-Chester Disease - complications
Female
Hemangioma - blood
Hemangioma - etiology
Hemangioma - pathology
Humans
Investigative Report
Male
Medicine
Medicine & Public Health
Middle Aged
POEMS Syndrome - blood
POEMS Syndrome - complications
Retrospective Studies
Skin Neoplasms - blood
Skin Neoplasms - etiology
Skin Neoplasms - pathology
Tumor Burden
Vascular Endothelial Growth Factor A - blood
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Summary:Background Glomeruloid haemangioma (GH) is considered a specific marker of POEMS syndrome, despite some published GH cases unrelated to POEMS syndrome. Objectives To present two cases with GH and atypical presentations of Erdheim-Chester disease (ECD) or POEMS syndrome, as well as a retrospective monocentric study of histologically-confirmed GH. Materials and methods Clinical, biological and histological data of the patients is presented. Results In addition to the two presented cases, 11 GH histologically-confirmed cases were retrospectively identified. Six patients were female (46.2%; 95 CI: 12–64.9) and median age was 54 years (31–85). For 11 patients (84.6%; 95 CI: 65–104.2), a diagnosis of POEMS syndrome was retained, one patient had autoimmune hepatitis, and another had ECD. GH was localised to the trunk in 10 cases (76.9%; 95 CI: 54–99) and the legs in the other three. The median number of haemangiomas in the cohort was three (SD: 3.08). Median level of VEGF was 1,490 (610–12,000) ng/mL. All immunohistochemical staining for human herpesvirus 8 (HHV-8) was negative. Conclusions Of the 13 cases of GH, of which two were not clear-cut POEMS syndrome, we report the first case of GH associated with ECD. In this cohort, all patients had high serum levels of VEGF but no in situ HHV-8 latent infection. We hypothesise that GH might be linked to a high level of VEGF in these two rare diseases.
ISSN:1952-4013
1952-4013
DOI:10.1684/ejd.2018.3451