Hassoun Nasreen, Agabrya Abed, Levy Yishai
Department Internal Medicine D, Rambam Health Care Campus and Rappaport Faculty of Medicine, Technion, Haifa, Israel.
Harefuah. 2011 Jul;150(7):574-7, 618, 617.
We present a case of a 47-year-old man with mediastinal cancer manifesting as a rash, myaLgia and muscle weakness, in addition to the typical laboratory test results and histological changes compatible with dermatomyositis. The skin and muscLe findings followed the relapse of cancer which responded well to a combination of chemo and radiation therapy. Disease prevaLence in Rambam MedicaL Center during the past decade is also described. Dermatmyositis is a myositis with cutaneous manifestations. In a significant percent of the cases it can occur as a paraneoplastic syndrome. It was described as coetisting with various cancers, mostly adenocarcinomas. The majority of the cases occur in males above the age of 45 years. Malignancy can occur simultaneously with dermatomyositis or up to 3 years thereafter. Ovarian cancer is an exception, for it can be diagnosed up to six years after dermatomyositis. Therefore, it is advised to perform cancer screening in adult patients with dermatomyositis. Screening shouLd incLude extensive laboratory and imaging work-up. However, the mechanisms underlying paraneoplastic dermatomyositis are not fully understood. A possible antigenic similarity between cancer cell popuLations and regenerating myoblasts is suggested; this can result in an autoimmune autoantibody-mediated response in genetically predisposed patients. Numerous laboratory and clinical characteristics are associated with a high risk of malignancy. An update on paraneoplastic dermatomyositis is provided.
我们报告一例47岁男性纵隔癌患者,除了具有与皮肌炎相符的典型实验室检查结果和组织学改变外,还表现为皮疹、肌痛和肌肉无力。皮肤和肌肉表现出现在癌症复发之后,化疗和放疗联合治疗对此反应良好。文中还描述了过去十年间兰巴姆医疗中心的疾病患病率。皮肌炎是一种伴有皮肤表现的肌炎。在相当一部分病例中,它可作为副肿瘤综合征出现。它被描述为与各种癌症同时存在,大多数为腺癌。大多数病例发生在45岁以上的男性。恶性肿瘤可与皮肌炎同时出现,或在其后长达3年出现。卵巢癌是个例外,因为在皮肌炎出现后长达6年才可诊断出卵巢癌。因此,建议对成年皮肌炎患者进行癌症筛查。筛查应包括全面的实验室检查和影像学检查评估。然而,副肿瘤性皮肌炎的潜在机制尚未完全明确。有人提出癌细胞群体与再生成肌细胞之间可能存在抗原相似性;这可能导致在具有遗传易感性的患者中出现自身免疫性自身抗体介导的反应。许多实验室和临床特征与高恶性肿瘤风险相关。本文提供了副肿瘤性皮肌炎的最新情况。