Department for Propaedeutics of Internal Medicine, Clinic of Rheumatology, Medical University, Plovdiv, Bulgaria.
Rheumatol Int. 2013 Jun;33(6):1597-9. doi: 10.1007/s00296-010-1715-8. Epub 2011 Jan 21.
The capillaroscopic pattern in paraneoplastic Raynaud's phenomenon (RP) has not been investigated systematically and is not well-defined. Here, we present three case reports of patients with paraneoplastic rheumatic conditions, manifested with severe secondary RP with emphasis upon capillaroscopic findings. The first patient is a 58-year-old male with known psoriasis and psoriatic arthritis, severe RP and scleroderma-like syndrome, resulting in a paraneoplastic syndrome in the context of a lung cancer. At capillaroscopic examination classic "scleroderma" pattern, an "early" type was found. The second patient is a 48-year-old woman with an abrupt onset of paraneoplastic dermatomyositis, severe RP, and a lung cancer. The capillaroscopic examination revealed frequent dilated and giant capillaries, hemorrhages and severe microvascular disarrangement-the so-called "scleroderma-like" pattern typical of the idiopathic forms of the disease. The third patient is a 56-year-old woman with paraneoplastic dermatomyositis, secondary RP, and thyroid cancer. The capillaroscopic examination showed dilated and giant capillaries, elongated capillaries, decreased mean capillary density with avascular areas, severe disarrangement, single hemorrhages, and clear evidence of neoangiogenesis. These capillaroscopic features characteristic of the "scleroderma-like" pattern are indistinguishable from those in idiopathic dermatomyositis like in the second case. Taken together, the cases illustrate the problem that capillaroscopic patterns in paraneoplastic RP in the context of scleroderma-like syndrome and dermatomyositis appear to be indistinguishable from the microvascular changes in the respective idiopathic rheumatic diseases.
副肿瘤性雷诺现象(RP)的毛细血管模式尚未得到系统研究,也没有明确定义。在这里,我们报告了三例副肿瘤性风湿性疾病患者的病例,这些疾病表现为严重的继发性 RP,并强调了毛细血管镜检查结果。
第一位患者是一名 58 岁男性,患有已知的银屑病和银屑病关节炎,严重的 RP 和硬皮病样综合征,导致肺癌相关的副肿瘤综合征。在毛细血管镜检查中发现了典型的“硬皮病”模式,属于“早期”类型。
第二位患者是一名 48 岁女性,突然出现副肿瘤性皮肌炎、严重的 RP 和肺癌。毛细血管镜检查显示频繁出现扩张和巨大毛细血管、出血和严重的微血管排列紊乱——所谓的“硬皮病样”模式,这是该疾病特发性形式的典型表现。
第三位患者是一名 56 岁女性,患有副肿瘤性皮肌炎、继发性 RP 和甲状腺癌。毛细血管镜检查显示扩张和巨大毛细血管、伸长的毛细血管、平均毛细血管密度降低伴有无血管区、严重的排列紊乱、单个出血和新血管生成的明确证据。这些毛细血管特征与特发性皮肌炎的“硬皮病样”模式特征相似,与第二位患者的特征无法区分。
综上所述,这些病例说明了在硬皮病样综合征和皮肌炎背景下的副肿瘤性 RP 的毛细血管模式与各自特发性风湿性疾病的微血管变化似乎无法区分的问题。