幼年皮肌炎74例临床分析

目的 分析幼年皮肌炎（juvenile dermatomyositis，JDM）患儿的临床资料，总结该病的临床特征及误诊原因，为本病临床诊治提供依据。方法 收集2015年1月至2018年12月郑州大学第一附属医院收治的74例JDM患儿的性别、发病年龄、首发症状、临床特征、实验室检查等资料进行回顾性分析，总结JDM的临床特征、误诊原因及治疗经验。结果 JDM女性多于男性，男女比例1∶1.39，发病年龄男女无差异；首发症状以皮疹（51例）最为多见，包括面部、眶周暗紫红色水肿性斑疹、Gottron征等；以肌无力、肌痛为首发症状者19例，以关节症状起病者4例。30例患儿首次就诊时误诊，误诊率40.54%，以皮疹为首发症状者误诊29例，分别误诊为湿疹（17例）、接触性皮炎（5例）、荨麻疹（3例）、过敏性紫癜（2例）、神经性皮炎（1例）、亚急性皮肤型红斑狼疮（1例）；1例患儿首诊时被误诊为心肌炎。近一半的患儿出现肌酸激酶（27例）、肌酸激酶同工酶（26例）、α-羟丁酸脱氢酶（35例）升高。52例患儿肌电图示肌源性损害。经糖皮质激素或联合免疫抑制剂治疗后患儿病情好转出院。结论 JDM临床表现多样，眶周暗紫红色水肿性斑疹和Gottron征对诊断意义较大，仔细查体和全面的辅助检查可减少漏诊误诊，治疗首选糖皮质激素和免疫抑制剂。

Clinical analysis of 74 cases of juvenile dermatomyositis

Objective To analyze the clinical data of children with juvenile dermatomyositis (JDM), summarize the clinical features and causes of misdiagnosis of the disease, and provide a basis for the clinical diagnosis and treatment of this disease. Methods The data of 74 children with JDM admitted to the First Affiliated Hospital of Zhengzhou University from January 2015 to December 2018, including gender, age of onset, initial symptom, clinical features and laboratory tests, were collected and retrospectively analyzed to summarize the clinical features, causes of misdiagnosis and treatment experience of JDM. Results There were more women than men in JDM, the ratio of male to female was 1:1.39, and there was no difference in the age of onset between men and women. The most common initial symptom was rash (51 cases), including facial and periorbital dark purple edematous rash, Gottron sign, etc. There were 19 cases with muscle weakness and myalgia as the first symptoms, and 4 cases with joint symptoms. 30 cases were misdiagnosed at the first visit, and the misdiagnosis rate was 40.54%. 29 cases with rash as the first symptom were misdiagnosed as eczema (17 cases), contact dermatitis (5 cases), urticaria (3 cases), allergic purpura (2 cases), neurodermatitis (1 case), subacute cutaneous lupus erythematosus (1 case). One child was misdiagnosed as myocarditis at the first diagnosis. Nearly half of the children had elevated creatine kinase (27 cases), creatine kinase isoenzyme (26 cases), and alpha-hydroxybutyrate dehydrogenase (35 cases). Electromyography showed myogenic damage in 52 children. The patients were discharged after treatment with glucocorticoid or combined immunosuppressive agents. Conclusions The clinical manifestations of JDM are diverse. Periorbital dark purple edematous rash and Gottron sign are of great significance for diagnosis. Careful physical examination and comprehensive auxiliary examination can reduce missed diagnosis and misdiagnosis. Glucocorticoids and immunosuppressants are the first choice for treatment.