皮肌炎合并免疫性血小板减少症1例  

Dermatomyositis Associated with Immune Thrombocytopenia: A Case Report

作　　者：陈妍静 李桐[1] 李晓红[1] 张雪毓 闫薇[1] CHEN Yanjing;LI Tong;LI Xiaohong;ZHANG Xueyu;YAN Wei(West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院,四川成都610041

出　　处：《中国皮肤性病学杂志》2024年第10期1158-1161,共4页The Chinese Journal of Dermatovenereology

摘　　要：患者男,48岁,背部紫红斑4个月余,累及面部伴肌肉疼痛2个月。查体:颈部肌力Ⅳ-级,左上臂肌力Ⅳ+级。皮肤科情况:面部、上臂、躯干散在紫红色斑片、鳞屑,双膝伸侧Gottron征。辅助检查示:抗核抗体1∶320(核仁型胞浆颗粒型),抗双链DNA抗体>300.00 IU/mL,抗U1-nRNP抗体/Sm抗体(+),抗核糖体P蛋白抗体(2+)。皮损组织病理示:表皮棘层轻度增厚,灶性基底细胞液化,血管周围中等量淋巴细胞浸润,似见黏液沉积。阿新蓝染色:真皮浅层胶原纤维间少量阳性。肌肉活检提示炎性肌病改变。住院期间,患者出现严重且顽固的血小板减少症,血小板特异性抗体阳性。诊断:皮肌炎合并免疫性血小板减少症。给予人免疫球蛋白治疗,病情改善。院外随访4个月余,患者血小板及其他实验室指标恢复正常。A 48-year-old male presented with purplish erythema on his back for over 4 months,involving the face and muscle pain for 2 months.Physical examination revealed gradeⅣ-strength in the neck muscles and gradeⅣ+strength in the left upper arm.Scattered purplish-red patches and scales on the face,upper arms and trunk,and Gottron′s sign on both knees.Laboratory tests showed ANA was 1∶320(nucleolar and cytoplasmic speckle pattern),anti-dsDNA>300.00 IU/mL,anti-U1-nuclear ribonucleoprotein/smithantibody was positive(+),and anti-ribosomal P protein antibody was positive(2+).Skin lesion histopathology revealed mild thickening of the epidermal layer,focal liquefaction of basal cells,moderate perivascular lymphocyte infiltration,and mucin deposition which was positive for alcian blue staining.A muscle biopsy showed inflammatory myopathy changes.During hospitalization,the patient developed severe and refractory thrombocytopenia with positive platelet-specific antibodies.The diagnosis of dermatomyositis associated with immune thrombocytopenia was made.Patient was treated with intravenous immunoglobulin and symptom improvement.After four months of follow-up,the patient′s platelets and other laboratory indicators returned.

关 键 词：皮肌炎 免疫性血小板减少 Gottron征 

分 类 号：R593.26[医药卫生—内科学] R558.2[医药卫生—临床医学]