幼年皮肌炎:诊断与管理。

Skinmed Pub Date : 2024-09-17 eCollection Date: 2024-01-01
Amina Aounallah, Sarra Saad, Nadia Ghariani Fetouri, Mohamed Denguezli
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摘要

幼年皮肌炎(JDM)是儿童自身免疫性慢性特发性炎症性肌病的主要病因1。我们的研究共涉及 6 名女孩和 1 名男孩,发病年龄中位数为 8.16 岁2 。2 名患者起病急。所有患者在确诊时都有皮肤表现,4 例患者出现近端肌肉无力。四名患者的肌酶升高,所有患者的肌电图均显示为肌病。6 名患者接受了口服皮质类固醇和其他系统治疗。三名患者的治疗效果良好,而另外两名患者则复发了。另外两名患者对皮质类固醇产生了耐药性,其中一例患者死亡。本研究强调了幼年皮肌炎的诊断特点和治疗方法。
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Juvenile Dermatomyositis: Diagnosis and Management.

Juvenile dermatomyositis (JDM) is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children.1 Seven patients with JDM found in the records from 1998-2019 of the Department of Dermatology Farhat Hached Hospital, Sousse, Tunisia. Our study concerned a total of six girls and one boy with a median age at disease onset of 8,16 years.2 The average time before diagnosis was 8,8 months. The onset of the disease was acute in 2 patients. All patients displayed skin manifestations at diagnosis, with proximal muscular weakness in 4 cases. Four patients had elevated muscle enzymes and all of them showed myopathic findings on electromyography. Oral corticosteroids were prescribed in 6 patients, in association with other systemic therapies. Three patients achieved a good outcome while two others relapsed. The two other patients showed corticosteroids resistance with a fatal outcome in one case. This study highlights the diagnostic features and management of juvenile dermatomyositis.

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