Lim Darosa, Landon-Cardinal Océane, Ellezam Benjamin, Belisle Annie, Genois Annie, Sirois Jennifer, Bourré-Tessier Josiane
Division of Dermatology, Department of Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada.
Division of Rheumatology, Department of Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada.
SAGE Open Med Case Rep. 2020 Dec 29;8:2050313X20984120. doi: 10.1177/2050313X20984120. eCollection 2020.
Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.
抗3-羟基-3-甲基戊二酰辅酶A还原酶（HMGCR）免疫介导的坏死性肌病是特发性炎性肌病的一种亚型，可能与他汀类药物暴露有关。其表现为严重的近端肌无力、肌酸激酶水平升高和肌纤维坏死。通常需要静脉注射免疫球蛋白和免疫抑制剂进行治疗。由于通常没有肌肉外表现，皮肤科医生对这个实体并不常见。我们报告了一例罕见的他汀类药物相关抗HMGCR免疫介导的坏死性肌病，伴有皮肌炎样皮肤特征。对于伴有严重近端肌无力、肌酸激酶水平高度升高且可能有他汀类药物暴露的皮肌炎样皮肤特征患者，应考虑抗HMGCR免疫介导的坏死性肌病的可能性。这表明肌肉活检和特定自身抗体检测对于准确诊断的重要性，以及显著的治疗意义。
