Patil Anil Kumar B, Prabhakar A T, Sivadasan Ajith, Alexander Mathew, Chacko Geeta
Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.
Neurol India. 2015 Jan-Feb;63(1):72-6. doi: 10.4103/0028-3886.152642.
Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing. The features were of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. She was pulsed with intravenous immunoglobulin, methylprednisolone, and cyclophosphamide and showed a good improvement. In the absence of widespread inflammatory response and classical histopathology findings, it is important to diagnose this condition as it shows a good response to aggressive and prolonged immunotherapy.
伴有管状毛细血管的坏死性肌病是慢性炎症性肌病的一种形式，其组织病理学表现为坏死性肌病、极少的细胞浸润和微血管病。一名30岁女性出现进行性肢体无力6个月，伴有皮肤色素沉着和雷诺现象。血清肌酸磷酸激酶为3990 u/L。肌肉活检显示坏死纤维、局灶性血管周围稀疏炎症/束周萎缩、肌内膜/肌外膜血管壁增厚伴管腔狭窄。其特征为伴有管状毛细血管/微血管病的炎症性坏死性肌病和神经病变。她接受了静脉注射免疫球蛋白、甲泼尼龙和环磷酰胺冲击治疗，病情有明显改善。在缺乏广泛炎症反应和典型组织病理学表现的情况下，诊断这种疾病很重要，因为它对积极且长期的免疫治疗反应良好。
