What is dermatomyositis?

皮肌炎是一种罕见的疾病，会引起肌肉炎症和皮疹. 它是引起肌肉炎症和肿胀的一组肌肉疾病之一. 它与其他肌肉疾病不同，因为它也会引起皮肤问题. 皮肌炎是用来描述肌肉和皮肤症状的术语.

它可以发生在任何年龄，但最常发生在50至70岁的成年人身上. 女性被诊断患有这种疾病的可能性是男性的两倍. 一些患有这种疾病的人还患有结缔组织疾病, such as lupus or rheumatoid arthritis.

What causes dermatomyositis?

The exact cause is not known, but possible causes include:

• Abnormal genes you are born with

• Cancer, especially in older people

• 自身免疫性疾病，一种导致人体免疫系统攻击自身组织的疾病

• 感染、药物或环境中的其他因素引发了疾病

What are the symptoms of dermatomyositis?

这些症状是由皮肤和肌肉血管的肿胀和炎症引起的, and can include:

• 暴露在阳光下的部位出现红色或紫色皮疹，可能会疼痛或发痒

• Red or purple swelling of the upper eyelids (heliotrope)

• 指节、肘部、膝盖和脚趾上的红色或紫色斑点(Gottron丘疹)

• 关节在寒冷的情况下会感到疼痛和苍白，温暖的时候会感觉好一些(雷诺现象)。

• Scaly, rough, dry skin, which can lead to hair thinning

• Swollen, red areas around the fingernails

• 由钙沉积引起的皮肤下硬块(钙质沉着症)

• Muscle weakness in the neck, hip, back, and shoulders

• Trouble swallowing and voice changes

• Tiredness, fever, and weight loss

• Muscle aches

• 由于肌肉无力，无法从椅子上起身或下床

有时肌肉无力也会扩散到心脏、胃肠道和肺部. This can cause breathing trouble and coughing. 成年人可能会有低烧，同时伴有肺部炎症和对光敏感.

How is dermatomyositis diagnosed?

这个过程从一个人的病史和身体检查开始. 医疗保健提供者会寻找潜在的疾病，比如癌症. Tests may also be done, such as:

• Blood tests. These are done to look for signs of muscle inflammation. 他们还检查自身免疫性疾病中形成的异常蛋白质. 最常见的血液检查包括肌酶肌酸激酶和抗核抗体.

• Electromyelogram (EMG). 这可以用来发现受影响肌肉的异常电活动.

• MRI. 这种测试使用大磁铁和一台电脑来寻找体内的炎症.

• Skin or muscle biopsy. 细小的组织被拿去用显微镜检查.

How is dermatomyositis treated?

治疗将取决于你的症状、年龄和整体健康状况. 这种病无法治愈，但症状是可以控制的. You may need more than one kind of treatment. And your treatment may need to be changed over time. Treatments include:

• Physical therapy. 特殊的运动有助于伸展和加强肌肉. Orthotics or assistive devices may be used.

• Skin treatment. 你可能需要避免暴露在阳光下，涂抹防晒霜来帮助防止皮疹. 你的医疗保健提供者可以用抗组胺药物或抗炎类固醇药膏治疗皮肤瘙痒皮疹.

• Anti-inflammatory medications. These are steroid drugs, or corticosteroids. They ease inflammation in the body. They may be given by mouth or through an IV.

• Immunosuppressive drugs. 这些药物会阻碍或减慢你身体的免疫系统. 这些药物包括硫唑嘌呤、甲氨蝶呤、环孢素A、环磷酰胺和他克莫司.

• Immunoglobulin. 如果你对其他治疗没有反应，这些药物可能会被给予. 它们是捐赠的血液制品，可以增强你身体的免疫系统. They are put directly into your bloodstream through an IV.

• Surgery. 如果皮肤下的钙沉积(钙质沉着症)变得疼痛或感染，可以通过手术去除.

Talk with your health care providers about the risks, benefits, and possible side effects of all medications.

Dermatomyositis in Children

What is dermatomyositis?

儿童皮肌炎描述了一组以慢性肌肉炎症为特征的肌肉疾病，随后是进行性肌肉无力.

Symptoms

• 在肌肉无力之前或伴随出现的皮疹(斑片状的、带蓝色/紫色或红色)

• Swelling in the affected area

• Calcium deposits

Diagnosis

需要仔细的临床评估来确定症状的原因.

Treatment

There is no cure for dermatomyositis, but you can treat the symptoms with medication, physical therapy, exercise, heat therapy and rest. 药物包括皮质类固醇、免疫抑制药物和局部软膏.

What are the complications of dermatomyositis?

皮肌炎患者可能出现的并发症包括肺部疾病, heart disease, or cancer. These can make treatment more difficult.

Living with dermatomyositis

如果你患有皮肌炎，你可能需要终生治疗. 尽可能多地了解这种疾病是很重要的. Work closely with your health care provider. 研究人员正在研究这种情况的原因和治疗方法. 随着时间的推移，这种疾病的早期诊断和治疗可能会有所改善.

When should I call my health care provider?

如果您的症状加重或发现新的症状，请通知您的医疗保健提供者.

Key points

• 皮肌炎是一种罕见的疾病，会导致肌肉无力和皮疹.

• 症状包括暴露在阳光下的皮肤和眼睑出现红色或紫色皮疹, calcium deposits under the skin, muscle weakness, and trouble talking or swallowing.

• 这种病无法治愈，但可以通过治疗来减轻症状.

• 并发症包括肺病、心脏病和癌症.

Next steps

帮助你从医疗保健提供者那里获得最大好处的建议:

• Before your visit, write down questions you want answered.

• 带一个人来帮你问问题，记住你的医生告诉你的.

• At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.

• 如果你有后续预约，写下日期、时间和目的.

• 知道如果你有问题如何联系你的供应商.