无肌病性皮肌炎

病史：患者男，51岁。6年前双眼脸原因不明出现紫红色水肿性斑片，并在其后的2年内，逐渐蔓延至整个颜面、颈部、上胸、肩胛及手背等处，无任何自觉症状，惟夏季日晒后感轻度刺痒。出现皮损以来无肌痛、肌无力、发热、关节痛，头晕及其他不适，家族中无同类疾病患者无药物过敏史。患病后曾在其他医院诊治，诊断为红斑狼疮，多形日光疹、扁平苷藓、面癣及皮肌炎，但从未行组织病理学及其他实验室检查。     

无肌病性皮肌炎一例

患者,女,21岁。面部、躯干、四肢皮肤红斑、丘疹伴脱发6年,皮损基本损害为红斑,双眼睑见明显水肿性紫红斑,其他部位皮损为弥漫性对称性暗紫红色斑,双手可见Gottron丘疹。肌力正常。皮损组织病理检查：表皮轻度角化过度,基底膜增厚,真皮浅层水肿,血管周围单一核细胞浸润。诊断：无肌病性皮肌炎。给予泼尼松联合甲氨蝶呤治疗2个月后皮损明显好转。     

无肌病性皮肌炎1例

患者女，60岁。因前额、眼睑出现水肿性红斑1年，近3个月加重且伴剧烈瘙痒．于2000年12月18日我科门诊以“血管萎缩性皮肤异色症”收入院。患者诉1年前，因染发后前额出现肿胀、红斑，双眼睑红肿，被诊断为染发引起的“接触性皮炎”，随即给予抗过敏药物治疗，不见好转．反而进行性加重。患者余无不适，体温正常。曾予泼尼松40mg／d治疗2个月，面、额部红斑消退，但停药后红斑再次出现。患者有“高血脂”病史2年，1年前曾行“右侧乳腺纤维瘤”手术。     

临床无肌病性皮肌炎的诊断及治疗

临床无肌病性皮肌炎被认为是皮肌炎的一种少见类型,是指具有皮肌炎的典型皮损,临床上不表现为肌无力,肌酶谱正常或仅轻度异常,分为无肌病性皮肌炎和低肌病性皮肌炎。部分患者与严重的肺部病变及恶性肿瘤相关,而部分严重患者对糖皮质激素治疗不敏感,故早期发现和正确认识该病并给予合理的干预,对改善患者预后非常重要。     

皮肌炎样皮炎还是无肌病性皮肌炎?——关于无肌病性皮肌炎命名的商榷

Pearson首次用“无肌病性皮肌炎(ADM)”来命名有皮肌炎的典型皮损而无肌肉损害的患者。然而大家对这一疾病的命名一直存在争议。复习大量文献后,笔者提出“皮肌炎样皮炎”这样的命名,并认为它是一种复杂的综合群,可以是皮肌炎的早期改变,也可以伴有严重的肺部损害或疾病或向红斑狼疮转归,让人忽略的是这样的皮炎还可以合并恶性肿瘤,是“副肿瘤综合征”的一种表现。对这类患者要加强随访,定期作肿瘤学检查和胸部放射学检查。     

无肌病性皮肌炎16例临床分析

目的：探讨无肌病性皮肌炎（ADM）的命名。方法：回顾性分析符合Euwer提出的ADM诊断标准的16例患者的临床资料。结果：16例患者中2例进展为皮肌炎,1例进展为慢性皮肤型红斑狼疮（CCLE）。间质性肺炎（ILD）发生率为68．8％,恶性肿瘤发生率为25．0％。结论：提出皮肌炎样皮炎的命名,并认为它是一种复杂的综合征,可以是皮肌炎的早期表现,也可以伴有严重的肺部损害或疾病向红斑狼疮转归,皮肌炎样皮炎可并发恶性肿瘤,因此需要行全身系统检查,并加强随访。     

儿童无肌病性皮肌炎1例

报告1例具有典型的皮肌炎皮疹而无肌肉受累的儿童无肌病性皮肌炎患者。结合文献对本病的临床表现、诊断及治疗进行讨论。本病的诊断需临床、病理与实验室检查相结合。     

无肌病性皮肌炎1例

报告1例无肌病性皮肌炎。患者女,55岁,眼睑红肿、面部头及皮红斑,伴瘙痒反复发生8年。发病以来无肌肉损害的临床表现,与肌肉损害有关的实验室检查及免疫学指标均正常,皮损的组织病理检查结果符合皮肌炎诊断。     

成功治愈1例无肌病性皮肌炎

无肌病性皮肤炎（amyopathic dermatomyositis,ADM)，是指具有典型的皮肌炎（DM）的皮损而无肌病变依据，且持续2年以上的病例。ADM较省见，约占皮肌炎病例的10%左右。对本病 的治疗和预后各家报道不一。     

关于无肌病性皮肌炎命名的商榷

拜读了曹华等“皮肌炎样皮炎还是无肌病性皮肌炎？——关于无肌病性皮肌炎命名的商榷”一文，又读到编者按“欢迎广大读者对此展开讨论”，故谈几个观点与作者及读者商榷。     

A systematic review of juvenile-onset clinically amyopathic dermatomyositis

BACKGROUND: Dermatomyositis (DM) presenting during childhood or adolescence classically encompasses hallmark cutaneous changes, proximal muscle weakness, and laboratory evidence of myositis. When cutaneous manifestations of DM are present without muscle weakness for > 6 months, the term 'clinically amyopathic DM' applies (syn. DM sine myositis). OBJECTIVES: To review the clinical and epidemiological features of published cases of juvenile-onset clinically amyopathic DM, with comparison with adult-onset clinically amyopathic DM and juvenile-onset classical DM. METHODS: Systematic review of the published literature. RESULTS: We identified 68 cases of juvenile-onset clinically amyopathic DM published during 1963-2006. The disease in 18 of 68 (26%) patients subsequently evolved to classical DM. Overall, the mean age at diagnosis was 10.8 years (range 2-17) with nearly equal male/female ratio and mean follow-up of 3.9 years. Among cases with diagnostic testing, 10 of 19 had a positive antinuclear antibody titre, two of nine had elevated erythrocyte sedimentation rate and two of 51 had elevated creatine kinase (CK). Of patients with normal CK, three of 22 had abnormal electromyography, one of 19 had abnormal muscle biopsy, and one of nine had abnormal magnetic resonance imaging. Calcinosis was reported in three of 68. No cases of severe vasculopathy (resulting in ulceration), interstitial lung disease or internal malignancy were reported. CONCLUSIONS: This review suggests a good prognosis for children with clinically amyopathic DM. A minority of patients with negative muscle enzymes had positive ancillary testing for myositis, and these patients rarely developed muscle weakness. Predictive factors for progression to classical DM were not identified. Symptomatic treatment of cutaneous involvement and close clinical monitoring may be an alternative to aggressive immunosuppression.     

皮肌炎/临床无肌病性皮肌炎患者血清中CADM-140抗体的检测和临床意义

目的 对皮肌炎（DM）/临床无肌病性皮肌炎（CADM）患者进行CADM-140抗体检测,探讨CADM-140抗体与临床特征间的联系。方法 采集38例DM（22例）/CADM（16例）患者血清,另外采集46例伴有肺间质病变的其他结缔组织病患者血清,包括8例多发性肌炎、15例系统性红斑狼疮、5例系统性硬化病、6例干燥综合征、6例混合性结缔组织病、6例特发性肺纤维化和5例正常对照者。以重组黑素瘤分化相关基因5（rMDA-5）为底物,通过ELISA检测患者血清中CADM-140抗体,比较CADM-140抗体阳性与阴性患者的临床特征。 结果 ①16例CADM和22例DM患者血清CADM-140抗体阳性例数分别为7例和2例,CADM患者阳性率（43.8%）显著高于DM（9.1%）（P ＜ 0.05）,46例伴有肺间质病变的其他结缔组织病患者及5例正常人均阴性;②CADM-140抗体阳性患者皮肤溃疡和坏死的发生率为8/9,红细胞沉降率为（40.8 ± 23.1） mm/1 h,CADM-140抗体阴性组分别为6.9%和（22.5 ± 16.8） mm/1 h,两组比较,P ＜ 0.01和 ＜ 0.05;CADM-140抗体阳性患者乳酸脱氢酶水平显著高于阴性组（分别为328.3 ± 104.2和241.1 ± 100.3 IU/L,P ＜ 0.05）,而肌酸激酶显著低于阴性组（分别为156.3 ± 260.8和1806.2 ± 3737.1 IU/L,P ＜ 0.05）;两组间抗核抗体阳性率和恶性肿瘤发生率的差异无统计学意义;③CADM-140抗体阳性患者不仅肺间质病变发生率显著高于阴性组（分别为9/9和48.3%,P ＜ 0.01）,而且急进型肺间质病变发生率也显著高于阴性组（分别为5/9和0,P ＜ 0.05）。阳性组肺高分辨率CT评分（122.9 ± 54.8）显著高于阴性组（70.0 ± 59.8）（P ＜ 0.05）。结论 通过检测CADM-140抗体不仅可以判断DM/CADM是否合并肺间质病变,还可能是伴发急进型肺间质病变的血清学标记,动态观察血清CADM-140抗体水平也许有助于预测肺间质病变病程。     

[开放获取] 临床无肌病性皮肌炎诊断标准的演进

【摘要】 目前皮肌炎诊断使用最广泛的标准是Bohan和Peter标准,该标准要求患者除典型皮疹外,还须满足至少2条肌炎表现才能诊断皮肌炎。临床无肌病性皮肌炎（CADM）因无肌炎表现而被排除在外,这使CADM的早期研究十分匮乏,不被临床医生所重视。另外,诊断CADM依赖于对皮疹的认识和皮损组织病理表现,因CADM早期皮疹表现不典型,往往被误诊为玫瑰痤疮、脂溢性皮炎、红斑狼疮等其他疾病,从而影响早期诊断和治疗以及对疾病预后的判断。本文梳理CADM的诊断标准,以利于其早期识别和诊断,也为开展相关临床研究提供依据。     

Clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia: The relation between the disease activity and the serum interleukin‐6 level

A 67‐year‐old woman exhibited chilblain‐like erythema on the bilateral auricles, erythema with hyperkeratosis on the sides of fingers and the bilateral elbows, and erythema on the extensor/flexor sides of the finger joints and the iliac regions. Fine crackles were audible on the dorsal side of the bilateral lower lung regions. We diagnosed clinically amyopathic dermatomyositis accompanied by rapidly progressive interstitial pneumonia. While we initiated immunosuppressive therapy, the respiratory failure showed no responses. We performed endotoxin adsorption therapy using polymyxin B‐immobilized fiber column direct hemoperfusion (PMX‐DHP). After the first PMX‐DHP session, the respiratory condition was slightly improved but it soon deteriorated. At the second PMX‐DHP session, subcutaneous and mediastinal emphysema was noted. The patient died, showing no improvement in the respiratory condition. We measured the levels of serum interleukin (IL)‐6, tumor necrosis factor (TNF)‐α and IL‐10 before and after PMX‐DHP and compared them with previously reported levels. The levels of TNF‐α and IL‐10 showed no or slight changes, but those of IL‐6 at the beginning of the treatment were higher compared with previous reports. After the first PMX‐DHP, the IL‐6 level decreased with slight improvement in respiratory condition. However, after the second PMX‐DHP, it increased markedly with exacerbation of the respiratory failure. The serum IL‐6 level is considered to be effective for the evaluation of the disease activity and prognosis under the treatment by PMX‐DHP.     

皮肌炎合并肺间质病变

为了解皮肌炎的肺间质病变，对36例皮肌炎患者进行了分析。结果：肺间质病变者8例，占22.2%。皮肌炎合并肺间质病变患者发热、咳嗽发生率高，分别为87.5%、62.5%。抗Jo-1、RNP抗体、ANA阳性率分别为57.1%，42.9%和57.1%，均高于不合并肺间质病变者。     

Malignancy and interstitial pneumonitis as fatal complications in dermatomyositis.

By sending questionnaires to the major dermatological clinics in Japan, we examined the outcome and causes of death in 182 cases of dermatomyositis (DM) observed between 1983 and 1987. The mortality rate in adults was 26.7%. Associated malignancy occurred in 47 cases (25.8% of adults), and 16 of them died of neoplasms. In contrast to 43 carcinomas, only 1 patient died of lymphoma. Associated interstitial pneumonitis (IP) appeared in 31 cases. IP started simultaneously or within 6 months after the diagnosis of DM in the majority of these cases. Of the 13 fatal cases due to IP, 8 died of respiratory failure within two months of the onset of lung disease. In the malignancy group, periungual erythema was noted in a higher proportion. Raynaud's phenomenon was more frequently noted in the IP group. DM overlapped with other collagen diseases in 14 cases.     

无肌炎性皮肌炎并发鳞状细胞癌致上腔静脉综合征1例

报告1例伴发上腔静脉综合征的无肌炎性皮肌炎。患者男,61岁。反复双眼睑、面部、胸部紫红斑近2年,无明显肌肉症状,且逐渐出现上腔静脉回流受阻的表现,经实验室、CT及淋巴结组织病理学检查确诊为无肌炎性皮肌炎并发纵隔鳞状细胞癌致上腔静脉综合征。