View CLG: Dermatomyositis

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Q: 什么是皮肌炎? 它有哪些类型?
皮肌炎是一种以肌肉无力和特异性皮肤红疹为特征的炎症性疾病。它被认为是一种自身免疫性疾病,其中身体的免疫系统错误地攻击自身组织。皮肌炎的确切原因尚不清楚,但可能与遗传因素、免疫系统异常和环境触发因素(如病毒感染)有关。

皮肌炎有几种类型,包括:
1. 经典型皮肌炎:这种类型影响成人和儿童,表现为肌肉无力和皮肤红疹。
2. 少年型皮肌炎:这种类型出现在儿童中,主要症状与经典型皮肌炎相同,但也可以包括皮下钙化(皮下钙质沉积)。
3. 无肌病性皮肌炎:也称为皮肌炎无肌炎,这种类型涉及典型的皮疹而没有肌肉无力。
4. 药物诱发型皮肌炎:一些药物可以触发类似皮肌炎的症状。
5. 癌症相关性皮肌炎:在一些成人中,皮肌炎与某些癌症相关联。

皮肌炎的管理通常包括使用抑制免疫系统的药物、物理治疗,以及在某些情况下,治疗任何潜在的癌症。
Q: 鉴别诊断皮肌炎需要什么患者数据和检查结果,包括症状、病史、体查、诊断检查等?
为了鉴别诊断皮肌炎,需要收集全面的病人信息和测试结果。以下是详细清单:
1. 症状:病人的症状至关重要。皮肌炎的常见症状包括:
• 皮疹,尤其是在眼睑、面颊、鼻子、背部和胸部,通常呈紫色或红色。
• 肌肉无力,特别是近躯干的肌肉。
• 关节痛。
• 疲劳。
2. 病史:
• 过去的医疗和家族自身免疫疾病史。
• 暴露于潜在触发因素的历史,如阳光暴露、新药物或最近的感染。
3. 体格检查:
• 检查皮肤是否有特征性皮疹(紫罗兰色皮疹,Gottron's丘疹)。
• 评估肌肉力量和关节受累情况。
4. 诊断测试:
• 血液检测:肌酶水平升高(如肌酸激酶(CK)、醛缩酶、乳酸脱氢酶(LDH)和天冬氨酸转氨酶(AST))。
• 抗体测试:存在特定自体抗体如Anti-Jo-1、Anti-Mi-2等与皮肌炎相关的抗体。
• 肌电图(EMG):检测肌肉中的异常电活动。
• 肌肉活检:寻找炎症和其他特征性肌肉组织变化。
• 影像检测:MRI可用于检测肌肉组织中的炎症和异常。
• 皮肤活检:确认皮肤学特征与皮肌炎一致。

这些组成部分共同帮助区分皮肌炎与其他症状相似的状况,如多发性肌炎、红斑狼疮和硬皮病。
Q: 患有皮肌炎的病人有哪些具体的病情、体查结果和诊断检查结果?
病史采集:在怀疑患有皮肌炎的患者中,病史通常会显示出典型症状,如肌肉逐渐无力,特别是肩部和臀部,以及特征性皮疹。皮疹可能包括面部和眼睑上的紫色或暗红色变色,有时在颈部和前胸(披肩征)或指关节上(Gottron's papules)也会出现。患者还可能报告吞咽困难、疲劳和关节痛。

体格检查:在体格检查期间,医生会寻找肌肉无力和皮肤异常的迹象。标志性征兆包括Gottron's papules(指关节上的隆起、鳞状斑点)、紫晶疹(眼周的紫色变色)和披肩征。通常会测试肌肉力量,观察从坐位起立或爬楼梯时的困难。

诊断测试:
1. 血液测试:常见的肌酶水平升高,如肌酸激酶(CK)、丙醛糖酶、天冬氨酸转氨酶(AST)和丙氨酸转氨酶(ALT)。包括抗Jo-1和其他特异性肌炎抗体的自身抗体测试可以支持诊断。
2. 肌肉活检:可以显示炎症、肌纤维坏死和与皮肌炎相关的特征性变化。
3. 肌电图(EMG):此测试可以检测到肌炎典型的肌肉异常电活动。
4. 影像学测试:MRI可用于评估肌肉中的炎症和损伤。
Q: 请提供一个患有皮肌炎的病历案例,包括病情询问结果、体查结果、各种鉴别诊断必需的检查结果?
患者信息:
姓名:简·多伊
年龄:42
性别:女

症状:
• 上臂和大腿的肌肉逐渐无力,几个月来加剧
• 吞咽困难
• 面部、颈部和手部,尤其是指关节上的皮肤出现皮疹
• 增加疲劳
病史:
• 无已知药物过敏
• 甲状腺功能减退,通过左甲状腺素控制
• 无类似症状的既往史
体格检查:
• 眼周出现紫罗兰色皮疹
• 手背出现戈特龙丘疹
• 近端肌群肌力为3/5
诊断测试:
• 血液检测:肌酸激酶(CK)、丙二醛酶及肝酶水平升高
• 肌电图(EMG):显示肌病性变化的异常
• 肌肉活检:显示肌纤维萎缩和炎症细胞,与皮肌炎相符
• 自身免疫组套:抗Jo-1抗体阳性
鉴别诊断:
• 皮肌炎
• 多发性肌炎
• 系统性红斑狼疮(SLE)
• 类风湿关节炎
Q: 请提供一个用一段话清楚描述皮肌炎的病历案例?
皮肌炎是一种罕见的炎症性疾病,其特征是肌肉无力和独特的皮疹。病例示例可能涉及一名45岁女性,她报告说肩部和臀部的肌肉无力逐渐加剧,导致其难以进行如爬楼梯或举起物体等活动。她还报告经历了紫罗兰色的眼睑皮疹(日晕疹)和指关节上的红色鳞状斑块(格特隆丘疹)。血液测试显示肌肉酶水平升高,表明有肌肉炎症,MRI显示与肌炎一致的肌肉水肿。皮肤活检确认了界面性皮炎,这是皮肌炎的典型表现。治疗包括使用高剂量的皮质类固醇和免疫抑制药物来控制她的症状。
Q: 为鉴别诊断皮肌炎,需要排除哪些疾病?请逐一列出,简单说明。
皮肌炎是一种炎症性疾病,其特征是肌肉无力和皮肤皮疹。在考虑其鉴别诊断时,应排除几种疾病:
1. 多发性肌炎 - 与皮肌炎相似,但缺乏典型的皮疹。
2. 系统性红斑狼疮(SLE) - 也可引起皮肤变化和肌肉无力。
3. 硬皮病 - 涉及皮肤增厚,也可影响肌肉。
4. 类风湿关节炎 - 虽主要影响关节,但也可以引起类似的系统性症状。
5. 混合性结缔组织病(MCTD) - 兼有红斑狼疮、硬皮病和多发性肌炎的特征。
6. 包涵体肌炎 - 一种肌肉疾病,导致老年人逐渐肌肉无力。
7. 病毒性肌炎 - 由病毒引起的肌肉炎症,可模仿皮肌炎中所见的肌肉无力。

这些条件与皮肌炎有重叠的症状,因此仔细的评估和检测对于准确诊断至关重要。

Bilingual Comparison

Q: What is dermatomyositis? Its types?
Dermatomyositis is an inflammatory disease marked by muscle weakness and a distinctive skin rash. It is considered a type of autoimmune disorder where the body's immune system mistakenly attacks its own tissues. The exact cause of dermatomyositis is unknown, but it may be related to genetic factors, immune system abnormalities, and environmental triggers such as viral infections.

There are several types of dermatomyositis, including:
1. Classic Dermatomyositis: This type affects both adults and children, presenting with muscle weakness and a skin rash.
2. Juvenile Dermatomyositis: This type occurs in children, characterized mainly by the same symptoms as classic dermatomyositis but can also include calcinosis (calcium deposits under the skin).
3. Amyopathic Dermatomyositis: Also known as dermatomyositis sine myositis, this type involves the typical skin rash without the muscle weakness.
4. Drug-induced Dermatomyositis: Some medications can trigger symptoms similar to those of dermatomyositis.
5. Cancer-associated Dermatomyositis: In some adults, dermatomyositis appears in association with certain cancers.

The management of dermatomyositis typically involves medications to suppress the immune system, physical therapy, and in some cases, treatment of any underlying cancer.
Q: What patient information and test results, including symptoms, medical history, physical exams, and diagnostic tests, are required to differentially diagnose dermatomyositis?
To differentially diagnose dermatomyositis, a comprehensive collection of patient information and test results is necessary. Here’s a detailed list:
1. Symptoms: The patient's symptoms are crucial. Common symptoms of dermatomyositis include:
• Skin rash, particularly on the eyelids, cheeks, nose, back, and chest, often purplish or red.
• Muscle weakness, especially in the proximal muscles (near the trunk).
• Joint pain.
• Fatigue.
2. Medical History:
• Past medical and family history of autoimmune diseases.
• History of exposure to potential triggers, such as sun exposure, new medications, or recent infections.
3. Physical Examination:
• Inspection of the skin for characteristic rashes (heliotrope rash, Gottron's papules).
• Assessment of muscle strength and joint involvement.
4. Diagnostic Tests:
• Blood tests: Elevated levels of muscle enzymes (such as creatine kinase (CK), aldolase, lactate dehydrogenase (LDH), and aspartate aminotransferase (AST)).
• Antibody tests: Presence of specific autoantibodies like Anti-Jo-1, Anti-Mi-2, and others associated with dermatomyositis.
• Electromyography (EMG): To detect abnormal electrical activity in muscles.
• Muscle biopsy: To look for inflammation and other characteristic muscle tissue changes.
• Imaging tests: MRI may be used to detect inflammation and abnormalities in muscle tissue.
• Skin biopsy: To confirm the presence of dermatologic features consistent with dermatomyositis.

These components together help differentiate dermatomyositis from other conditions with similar symptoms, such as polymyositis, lupus, and scleroderma.
Q: What are the specific results from medical history taking, physical examinations, and diagnostic tests for a patient with dermatomyositis?
Medical History Taking: In patients suspected of having dermatomyositis, the medical history often reveals characteristic symptoms such as progressive muscle weakness, particularly in the shoulders and hips, and a distinctive skin rash. The rash may include a violet or dusky red coloration on the face and eyelids, and sometimes on the neck and anterior chest (shawl sign) or over the knuckles (Gottron's papules). Patients may also report difficulties swallowing, fatigue, and joint pain.

Physical Examination: During the physical examination, a doctor will look for signs of muscle weakness and skin abnormalities. The hallmark signs include Gottron's papules (raised, scaly bumps over the knuckles), a heliotrope rash (a purplish discoloration around the eyes), and the shawl sign. Muscle strength is tested, typically observing difficulties in rising from a seated position or climbing stairs.

Diagnostic Tests:
1. Blood Tests: Elevated levels of muscle enzymes such as creatine kinase (CK), aldolase, aspartate transaminase (AST), and alanine transaminase (ALT) are common. Autoantibody tests, including anti-Jo-1 and other myositis-specific antibodies, can support the diagnosis.
2. Muscle Biopsy: This can reveal inflammation, muscle fiber necrosis, and characteristic changes associated with dermatomyositis.
3. Electromyography (EMG): This test can detect abnormal electrical activity in muscles that is typical in myositis.
4. Imaging Tests: MRI may be used to assess inflammation and damage in muscles.
Q: Please provide an example medical record containing symptoms, medical history, physical examination, and results of diagnostic tests, which is required for a differential diagnosis for a patient with dermatomyositis?
Patient Information:
Name: Jane Doe
Age: 42
Gender: Female

Symptoms:
• Progressive muscle weakness in the upper arms and thighs, onset over months
• Difficulty swallowing
• Skin rash on the face, neck, and hands, particularly over the knuckles
• Increased fatigue
Medical History:
• No known drug allergies
• Hypothyroidism, controlled with levothyroxine
• No prior history of similar symptoms
Physical Examination:
• Heliotrope rash around the eyes
• Gottron's papules on the dorsal hands
• Muscle strength 3/5 in proximal muscle groups
Diagnostic Tests:
• Blood tests: Elevated levels of creatine kinase (CK), aldolase, and liver enzymes
• Electromyography (EMG): Abnormalities suggesting myopathic changes
• Muscle biopsy: Evidence of muscle fiber atrophy and inflammatory cells, compatible with dermatomyositis
• Autoimmune panel: Positive anti-Jo-1 antibody
Differential Diagnosis:
• Dermatomyositis
• Polymyositis
• Systemic lupus erythematosus (SLE)
• Rheumatoid arthritis
Q: Please provide a one-paragraph patient case example of dermatomyositis?
Dermatomyositis is a rare inflammatory disease characterized by muscle weakness and a distinctive skin rash. An example of a patient case could involve a 45-year-old female presenting with progressive muscle weakness in her shoulders and hips, making activities like climbing stairs or lifting objects difficult. She also reports experiencing a heliotrope rash (a purplish discoloration) on her eyelids and Gottron's papules (red, scaly patches) over her knuckles. Blood tests reveal elevated muscle enzymes, indicating muscle inflammation, and an MRI shows muscle edema consistent with myositis. A skin biopsy confirms interface dermatitis, typical of dermatomyositis. Treatment includes high-dose corticosteroids and immunosuppressive drugs to control her symptoms.
Q: Briefly describe the diseases that should be ruled out in differential diagnosis of dermatomyositis?
Dermatomyositis is an inflammatory disease marked by muscle weakness and skin rash. When considering its differential diagnosis, several conditions should be ruled out:
1. Polymyositis - Similar to dermatomyositis but lacks the characteristic skin rash.
2. Systemic lupus erythematosus (SLE) - Can also cause skin changes and muscle weakness.
3. Scleroderma - Involves skin thickening and can also affect muscles.
4. Rheumatoid arthritis - While primarily affecting joints, it can also cause similar systemic symptoms.
5. Mixed connective tissue disease (MCTD) - This shares features of lupus, scleroderma, and polymyositis.
6. Inclusion body myositis - A muscle disease that leads to progressive muscle weakness, primarily in older adults.
7. Viral myositis - Muscle inflammation caused by a virus, which can mimic the muscle weakness seen in dermatomyositis.

These conditions share overlapping symptoms with dermatomyositis, making careful evaluation and testing crucial for accurate diagnosis.


ID: g5886844
Type: aiknowledge
UMLS CUI: C0011633
Status: shared
Created: 2024-06-01
By: aj_elhsi_org
Updated: 2024-09-07
By: aj_elhsi_org


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