Dermatomyositis Leading to Necrotizing Vasculitis: A Perfect Response to Applied Therapy

Mahmood Akbaryan, F. Darabi, Z. Soltani
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引用次数: 3

Abstract

Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Muscle weakness and skin lesions including heliotrope rash, shawl sign and Gottron’s papules are the most common symptoms. A biopsy (muscle or skin) is always the most reliable method for diagnosis. Corticosteroids in association with immunosuppressive agents are used as standard treatment. The patient was a 30 years old woman who got involved with dermatomyositis for 10 years. She has been under therapy with Methotrexate, Prednisolon and Azathioprine until she came to us suffering from progressive skin lesions. Experiments and examinations were normal except the lesions and detected lipoatrophy. Because of immune cells infiltration and observations necrotizing vasculitis was diagnosed. After three month of high dose prednisolon and intravenous cyclophosphamide therapy the lesions vanished remarkable. True and immediate diagnosis gives physicians the chance not only to assess the best treatment but have adequate time to apply the procedure. However shortening the therapy and diminishing morbidity of the disease need more investigations and efforts.
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皮肌炎导致坏死性血管炎:应用治疗的完美反应
皮肌炎是一种引起皮肤和肌肉并发症的特发性炎症性肌病。伦理学还没有被很好地理解。释放的细胞因子包括干扰素和白细胞介素被认为在皮肤或肌肉中引起炎症反应。最常见的症状是肌肉无力和皮肤病变,包括日光状皮疹、披肩征和Gottron丘疹。活检(肌肉或皮肤)总是最可靠的诊断方法。皮质类固醇联合免疫抑制剂被用作标准治疗。患者为30岁女性,患皮肌炎已有10年。她一直在接受甲氨蝶呤,泼尼松龙和硫唑嘌呤治疗,直到她来我们这里患有进行性皮肤病变。除病变及脂肪萎缩外,实验及检查均正常。经免疫细胞浸润及观察,诊断为坏死性血管炎。大剂量泼尼松龙和静脉环磷酰胺治疗三个月后,病变明显消失。真实和及时的诊断不仅使医生有机会评估最佳治疗方案,而且有足够的时间应用该程序。然而,缩短治疗时间和降低发病率需要更多的研究和努力。
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