Ofori Emmanuel, Ramai Daryl, Ona Mel, Reddy Madhavi
Department of Gastroenterology and Hepatology, The Brooklyn Hospital Center, Academic Affiliate of The Icahn School of Medicine at Mount Sinai, Clinical Affiliate of The Mount Sinai Hospital, 121 Dekalb Avenue, Brooklyn, NY 11201, USA.
Department of Anatomical Sciences, St. George's University School of Medicine, True Blue, Grenada, WI.
Gastroenterology Res. 2017 Aug;10(4):251-254. doi: 10.14740/gr841w. Epub 2017 Aug 31.
Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron's papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer. We report a 72-year-old female with renal clear cell carcinoma, breast cancer, and papillary serous carcinoma of the uterus, who presented with progressive weakness, 30 pound weight loss, and dysphagia over the past 3 months. She also reported difficulty in ambulating, facial rash, and inability to extend her fingers. Left bicep muscle biopsy and staining with nicotinamide adenine dinucleotide tetrazolium reductase (NADH-TR) identified frequent myofibers with targetoid-like changes consistent with DM. Despite immunosuppression with intravenous corticosteroids, she did not respond to the treatment and a percutaneous gastrostomy tube was placed for enteral feeding.
皮肌炎(DM)是一种罕见的自身免疫性疾病,主要影响50至60岁的女性。DM急性或渐进性起病,表现为无痛性近端骨骼肌无力,可伴有眼眶周围的向阳疹和手部伸侧的Gottron丘疹。虽然DM的病理生理学仍不清楚,但已有报道称存在异常的T细胞和B细胞免疫活性。DM与恶性肿瘤有关,已被证明可在癌症诊断之前、同时或之后出现。我们报告一例72岁女性,患有肾透明细胞癌、乳腺癌和子宫乳头状浆液性癌,在过去3个月中出现进行性肌无力、体重减轻30磅和吞咽困难。她还报告行走困难、面部皮疹以及无法伸直手指。左二头肌肌肉活检并用烟酰胺腺嘌呤二核苷酸四唑还原酶(NADH-TR)染色,发现频繁出现与DM一致的靶样改变的肌纤维。尽管静脉注射皮质类固醇进行免疫抑制治疗,但她对治疗无反应,遂放置经皮胃造瘘管进行肠内喂养。