Skin sclerosis as a manifestation of POEMS syndrome

Skin sclerosis as a manifestation of POEMS syndrome
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皮肤硬化是 POEMS 综合征的表现

DOI:
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发表时间:
2012
期刊:
Journal of dermatology (Print)
影响因子:
--
通讯作者:
K. Takehara
K. Takehara
中科院分区:
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文献类型:
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作者:
M. Hasegawa;Hidemitsu Orito;Keiko Yamamoto;T. Matsushita;Y. Hamaguchi;M. Fujimoto;K. Takehara

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我们报告一例64岁男性POEMS(多发性神经病变,器官肿大,内分泌病变,单克隆γ病变和皮肤变化)综合征,以前被误诊为系统性硬化症。他有POEMS综合征的典型症状,但存在皮肤硬化、手指挛缩和色素沉着,与系统性硬化症相似。包括本病例讨论的患者在内的10例患者在1990年至2011年间来我科就诊。其中5例患者有皮肤硬化。因此,我们比较POEMS综合征和系统性硬化症的皮肤病变和临床/实验室特征,试图区分这些疾病。根据皮肤和实验室检查结果,血管瘤或多毛的存在表明POEMS综合征。相比之下,系统性硬化症特异性自身抗体、甲襞出血、指溃疡/指凹陷疤痕或毛细血管扩张的存在高度提示系统性硬化症。据我们所知,这是第一篇详细讨论POEMS综合征与系统性硬化症鉴别的报道。
We report a case of a 64‐year‐old man with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome that had been previously misdiagnosed as systemic sclerosis. He had typical symptoms of POEMS syndrome, however, the existence of skin sclerosis, contracture of fingers and pigmentation were similar to that of systemic sclerosis. Ten patients, including the patient discussed in this case, visited our department between 1990 and 2011. Among them, five patients had skin sclerosis. Therefore, we compared skin lesions and clinical/laboratory features of POEMS syndrome and systemic sclerosis in an attempt to distinguish these disorders. Regarding the cutaneous and laboratory findings, the existence of hemangioma or hypertrichosis is indicative of POEMS syndrome. By contrast, the existence of systemic sclerosis‐specific autoantibodies, nail fold bleeding, digital ulcer/digital pitting scar or telangiectasia is highly suggestive of systemic sclerosis. To our knowledge, this is the first report to discuss in detail the differentiation between POEMS syndrome and systemic sclerosis.
DOI: --
发表时间: 1993-12
期刊: The Journal of rheumatology
影响因子: --
作者:
P. Clements;P. Lachenbruch;J. Seibold;B. Zee;V. Steen;P. Brennan;A. Silman;N. Allegar;J. Varga;M. Massa
通讯作者: P. Clements;P. Lachenbruch;J. Seibold;B. Zee;V. Steen;P. Brennan;A. Silman;N. Allegar;J. Varga;M. Massa