Chaouch N, Mjid M, Zarrouk M, Rouhou S C, Ammous I, Hantous S, Racil H, Chabbou A
Service de pneumologie, pavillon 2, hôpital A.-Mami, 2080 Ariana, Tunisie.
Rev Mal Respir. 2011 Sep;28(7):924-7. doi: 10.1016/j.rmr.2011.06.003. Epub 2011 Jul 23.
Erasmus' syndrome involves the association of systemic scleroderma (SS) and exposure to silica. Silicosis may precede the SS but the latter may be the presentation, in which case a history of exposure to silica should be sought as part of the diagnosis.
A 46-year-old man with history of pulmonary tuberculosis presented with dyspnoea and dysphagea. Clinical examination revealed thickening of the facial skin with a pointed nose, erythema and telangiectasia, Raynaud's syndrome and sclerodactyly. A thoracic CT scan revealed bilateral, fibrotic, pseudo-tumoural masses. Antinuclear antibodies, anti-topoisomerase 1 and antihistone were positive.
The clinical presentation of Erasmus' syndrome associating systemic scleroderma and pulmonary pseudo-tumours may pose a problem of differential diagnosis from lung cancer. This condition requires regular clinical and radiological monitoring, particularly as both scleroderma and silicosis increase the risk of lung cancer.
伊拉斯谟综合征涉及系统性硬化症(SS)与接触二氧化硅。矽肺可能先于系统性硬化症出现,但后者也可能是首发表现,在这种情况下,作为诊断的一部分,应询问接触二氧化硅的病史。
一名有肺结核病史的46岁男性出现呼吸困难和吞咽困难。临床检查发现面部皮肤增厚,鼻尖尖锐,有红斑和毛细血管扩张,雷诺综合征和指端硬化。胸部CT扫描显示双侧纤维化假瘤样肿块。抗核抗体、抗拓扑异构酶1和抗组蛋白均呈阳性。
伊拉斯谟综合征合并系统性硬化症和肺部假瘤的临床表现可能会带来与肺癌鉴别诊断的问题。这种情况需要定期进行临床和放射学监测,特别是因为系统性硬化症和矽肺都会增加患肺癌的风险。