Department of Pathology, Limoges, France.
Department of Neurology, National Referral Center for 'Rare Peripheral Neuropathies', Limoges, France.
J Neuropathol Exp Neurol. 2020 May 1;79(5):542-550. doi: 10.1093/jnen/nlaa021.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome is a rare systemic disorder linked to plasma cell dyscrasia and is related to elevation of vascular endothelial growth factor (VEGF). Diagnosis is still challenging and pathophysiology unclear. Because VEGF drives neovascularization, we investigated skin and nerve vascularization in 6 patients with POEMS syndrome compared with 5 control groups of polyneuropathies and healthy subjects (n = 104) from the University Hospital of Limoges between 2009 and 2018. We evaluated loss of small and large fibers in these patients. Skin and nerve vascularization were quantified manually on immunofluorescence using vessel staining (anti-α-SMA antibody). Dermal vascularization was significantly higher in POEMS syndrome than in other groups, but unrelated to loss of small fibers and VEGF. Perineurial vascularization was higher in POEMS syndrome than in healthy controls, and was related to loss of large fibers and VEGF level. Our study highlights the existence of neovascularization in skin of patients with this rare disorder. These data suggest that skin neovascularization could be an additional biomarker to help in the diagnosis and understanding of POEMS syndrome. Moreover, nerve neovascularization, driven by VEGF overexpression, may play a role in the pathophysiology of large fiber loss in this condition.
POEMS 综合征是一种罕见的系统性疾病，与浆细胞异常有关，与血管内皮生长因子 (VEGF) 的升高有关。其诊断仍然具有挑战性，发病机制尚不清楚。由于 VEGF 可促进新生血管形成，我们研究了 6 名 POEMS 综合征患者的皮肤和神经血管化情况，并与 2009 年至 2018 年期间来自利摩日大学医院的 5 组其他神经病变对照组和健康对照组（n=104）进行了比较。我们评估了这些患者的小纤维和大纤维的丧失情况。在免疫荧光染色上使用血管染色（抗-α-SMA 抗体）进行手动评估皮肤和神经血管化。POEMS 综合征患者的皮肤血管化明显高于其他组，但与小纤维丧失和 VEGF 无关。POEMS 综合征患者的神经外膜血管化高于健康对照组，与大纤维丧失和 VEGF 水平有关。我们的研究强调了这种罕见疾病患者皮肤中存在新生血管形成的现象。这些数据表明，皮肤新生血管形成可能是一种额外的生物标志物，有助于诊断和了解 POEMS 综合征。此外，VEGF 过度表达引起的神经新生血管化可能在该疾病大纤维丧失的发病机制中发挥作用。
