Akbaryan Mahmood, Darabi Farideh, Soltani Zahra
Professor of Medicine, Rheumathology Research Centre, Tehran University of Medical Sciences, Tehran, Iran.
Fellowship of Rheumatology, Rheumatologic Research Center, Tehran University of Medical Science, Tehran, Iran.
Int J Biomed Sci. 2016 Dec;12(4):125-129.
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.
皮肌炎是一种导致皮肤和肌肉并发症的特发性炎性肌病。其病因尚未完全明确。包括干扰素和白细胞介素在内的释放的细胞因子被认为会在皮肤或肌肉中引发炎症反应。肌无力和皮肤病变,包括向阳疹、披肩征和Gottron丘疹是最常见的症状。活检(肌肉或皮肤)始终是最可靠的诊断方法。皮质类固醇与免疫抑制剂联合使用作为标准治疗方法。该患者为一名30岁女性,患皮肌炎10年。在前来我们这里就诊前,她一直在接受甲氨蝶呤、泼尼松龙和硫唑嘌呤治疗,当时她正遭受进行性皮肤病变。除了病变和检测到的脂肪萎缩外,各项实验和检查均正常。由于免疫细胞浸润并观察到坏死性血管炎,故诊断为此病。经过三个月的大剂量泼尼松龙和静脉注射环磷酰胺治疗后,病变明显消失。准确及时的诊断不仅让医生有机会评估最佳治疗方案,而且有足够的时间实施该方案。然而,缩短治疗时间并降低该病的发病率还需要更多的研究和努力。