AESOP综合征:POEMS综合征的罕见早期指标。

IF 2.3 Q2 CLINICAL NEUROLOGY
Joumana Freiha, Clare Lodwick, Heather Roche, Chinar Osman
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引用次数: 0

摘要

腺病和广泛的皮肤斑块覆盖浆细胞瘤(AESOP)综合征是一种罕见的疾病,其特征是皮肤上出现红斑-紫色斑块,并伴有潜在的孤立浆细胞瘤。它可能为多神经病变、器官肿大、内分泌病变、单克隆蛋白、皮肤变化(POEMS)综合征(一种罕见的由浆细胞病变引起的多系统疾病)的早期诊断提供线索。我们描述一个62岁的男人感觉运动周围神经病变,无意体重减轻和持续皮疹。诊断结果包括血管内皮生长因子升高,免疫球蛋白A kappa副蛋白和皮肤/胸骨活检,导致AESOP和POEMS综合征的诊断。该病例强调了早期识别AESOP综合征作为早期诊断POEMS综合征的潜在救生线索的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
AESOP syndrome: a rare and early indicator of POEMS syndrome.

Adenopathy and an extensive skin patch overlying a plasmacytoma (AESOP) syndrome is a rare condition characterised by an erythemato-violaceous patch on the skin, accompanied by an underlying solitary plasmacytoma. It may give an early clue for the diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome, an uncommon multisystem disorder driven by plasma cell dyscrasia. We describe a 62-year-old man with sensorimotor peripheral neuropathy, unintentional weight loss and a persistent skin rash. Diagnostic findings, including elevated vascular endothelial growth factor, an immunoglobulin A kappa paraprotein and a skin/sternal biopsy, led to the diagnosis of AESOP and POEMS syndrome. This case underscores the importance of early recognition of AESOP syndrome as a potential lifesaving clue for the early diagnosis of POEMS syndrome.

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来源期刊
PRACTICAL NEUROLOGY
PRACTICAL NEUROLOGY Medicine-Neurology (clinical)
CiteScore
3.70
自引率
3.60%
发文量
113
期刊介绍: The essential point of Practical Neurology is that it is practical in the sense of being useful for everyone who sees neurological patients and who wants to keep up to date, and safe, in managing them. In other words this is a journal for jobbing neurologists - which most of us are for at least part of our time - who plough through the tension headaches and funny turns week in and week out. Primary research literature potentially relevant to routine clinical practice is far too much for any neurologist to read, let alone understand, critically appraise and assimilate. Therefore, if research is to influence clinical practice appropriately and quickly it has to be digested and provided to neurologists in an informative and convenient way.
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