Hernández Aragüés I, Pulido Pérez A, Ciudad Blanco C, Parra Blanco V, Suárez Fernández R
Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, España.
Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, España.
Actas Dermosifiliogr. 2017 Mar;108(2):e15-e19. doi: 10.1016/j.ad.2015.10.020. Epub 2016 May 18.
POEMS syndrome is a paraneoplastic manifestation associated with hematopoietic disorders such as multiple myeloma and Castleman disease. POEMS is an acronym for the main clinical features of the syndrome, namely, Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin abnormalities. Glomeruloid hemangiomas are considered to be a specific clinical marker of POEMS syndrome. However, while they are not pathognomonic, their presence should raise suspicion of this syndrome or alert clinicians to its possible future development, as these lesions can appear years before the onset of the syndrome. We report the cases of 2 women with plasma cell dyscrasias and sudden onset of lesions with a vascular appearance and histologic findings consistent with glomeruloid hemangioma. Recognition of this vascular tumor is important for the early diagnosis of POEMS syndrome.
POEMS综合征是一种与造血系统疾病相关的副肿瘤表现,如多发性骨髓瘤和Castleman病。POEMS是该综合征主要临床特征的首字母缩写,即多发性神经病、器官肿大、内分泌病、M蛋白和皮肤异常。肾小球样血管瘤被认为是POEMS综合征的一种特异性临床标志物。然而,虽然它们并非具有诊断意义,但它们的存在应引起对该综合征的怀疑,或提醒临床医生注意其未来可能的发展,因为这些病变可能在综合征发作前数年就出现。我们报告了2例患有浆细胞发育异常且突然出现具有血管外观和组织学表现符合肾小球样血管瘤的病变的女性病例。认识这种血管肿瘤对POEMS综合征的早期诊断很重要。