Syndrome POEMS révélé par un aspect de sclérodermie
Syndrome POEMS révélé par un aspect de sclérodermie
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硬皮病方面的综合症 POEMS révélé
DOI：
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发表时间：
2000
影响因子：
0.9
通讯作者：
M. Géniaux
中科院分区：
文献类型：
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作者：
P. Toussaint;V. Sibaud;L. Labbé;M. Géniaux
背景技术背景：POEMS综合征是一种罕见的浆细胞恶液质形式，其特征在于多发性神经病、器官肿大、内分泌病、单克隆成分和皮肤变化的各种相关性。最常见的皮肤变化，如色素沉着过度、皮肤增生、硬皮病样皮肤增厚和血管瘤不是特异性的，而是几乎恒定的。然而，他们很少孤立。病例报告：一位57岁的白人女性，有6个月的病史，双手和双脚皮肤增厚，雷诺现象和面部毛细血管扩张。体格检查提示肝肿大、感觉运动性周围神经病变（电生理评估为脱髓鞘型）体征和踝关节水肿。最初的实验室检查显示血小板计数为900 000/mm（3），这是一种单克隆IgG λ γ病。浆细胞轻微增加（骨髓元素10 p.100），全骨骼X线片显示无局灶性骨质增生或溶解性病变。诊断为POEMS综合征。患者接受他莫昔芬、甲基强的松龙和血浆置换治疗，多发性神经病或皮肤变化未改善。讨论：我们的病人符合POEMS综合征的标准。这里最典型的特征是硬皮病样皮肤变化，这已被其他作者所认识。但是，在本病例中，雷诺现象，皮肤增厚和面部毛细血管扩张在诊断前6个月就已存在，最初提示系统性硬皮病，经组织学证实。POEMS综合征的发病机制可能被认为是促炎细胞因子网络的显著激活的结果，但血清血管内皮生长因子（VEGF）水平的增加可以很好地解释其他表现，如皮肤增厚。
BACKGROUND: POEMS syndrome is a rare form of plasma-cell dyscrasia characterized by the various association of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes. The most frequent skin changes such as hyperpigmentation, hypertrichosis, scleroderma-like skin thickening and angiomas are not pathognomonic but nearly constant. However, they are rarely isolated. CASE REPORT: A 57-year-old Caucasian woman presented with a 6 month history of skin thickening on both hands and feet, Raynaud's phenomenon and facial telangiectasias. Physical examination on presentation revealed hepatomegaly, signs of a sensorimotor peripheral neuropathy which was demyelinating in type on electrophysiological assessment, and ankle edemas. Initial laboratory investigations revealed a platelet count of 900 000/mm(3), a monoclonal IgG lambda gammapathy. Plasma-cells were slightly increased (10 p. 100 of marrow elements) and full skeletal radiographs showed no focal osteosclerotic or lytic lesion. A diagnosis of POEMS syndrome was made. The patient was treated with tamoxifen, methylprednisolone and plasmapheresis without improvement in polyneuropathy or in skin changes. DISCUSSION: Our patient satisfied the criteria for POEMS syndrome. The most typical feature here was the scleroderma-like skin change, which has been recognized by other authors. But, in the present case, Raynaud's phenomenon, skin thickening and facial telangiectasias were present 6 months before the diagnosis, and initially suggestive of systemic scleroderma, confirmed histologically. The pathogenesis of POEMS syndrome might be regarded as the result of a marked activation of the proinflammatory cytokine network, but an increase in serum Vascular Endothelial Growth Factor (VEGF) levels could well account for other manifestations such as skin thickening.