160例皮肌炎临床分析

目的探讨皮肌炎的临床特点。方法对160例皮肌炎临床表现及实验室检查结果进行分析。结果发病年龄(14~60)岁患者占74.38%;首发症状以单独皮疹为主(50%);皮肤损害以上眼睑为中心的特征性水肿性紫红斑多见(72.5%);肌肉受累以肌无力多见(95%),以四肢近端为主。内脏受累:心脏(52.5%)、肺部(20.6%)、消化道(21.9%),肾脏损害轻微。5%患者合并恶性肿瘤。乳酸脱氢酶(LDH)、天冬氨酸转氨酶(AST)、丙氨酸转氨酶(ALT)、肌酸激酶(CK)、阳性率分别84.31%、73.87%、62.50%和44.35%。治疗以糖皮质类固醇激素为主。结论了解皮肌炎的发病特点,有利于临床中对皮肌炎的防控。     

皮肌炎合并间质性肺疾病患者的临床特点

间质性肺疾病(interstitial lungdisease,ILD)是皮肌炎的常见并发症^[1],并发率高达5%-46%^[2].合并ILD的皮肌炎患者预后差,治疗困难,死亡率高.部分患者肺部损害先于皮肤、肌肉症状出现,容易造成误诊,因此应重视皮肌炎的肺部损害.我们分析了27例皮肌炎-ILD患者的临床特点和肺部表现,以提高对ILD的认识.     

皮肌炎肺损害26例分析

目的观察皮肌炎合并间质性肺损害的临床特点,提高临床医生对皮肌炎肺损害的认识。方法回顾性分析1993年1月~2004年12月本科皮肌炎合并肺损害患者的一般资料、诊断、治疗及转归情况。结果患者的临床表现以发热、咳嗽、咳痰、呼吸困难、胸痛为主要症状,皮肌炎肺损害症状不典型,本组26例中误诊8例,误诊率31%,死亡8例,病死率31%。结论皮肌炎肺损害症状不典型,体征不明显,易误诊、漏诊,如不及时诊治,常会导致严重后果。     

98例皮肌炎患者临床分析

<正>皮肌炎(DM)是一种自身免疫性结缔组织病,该病典型症状是肌肉炎症和皮肤损害,可累及多系统病变,如肺部、关节及心脏等,部分患者还可伴发恶性肿瘤。现将我科近年收治的98例皮肌炎患者的临床资料进行回顾性分析,旨在探讨皮肌炎的首发症状、临床表现、实验室检查等特点,分析易误诊的原因和合     

皮肌炎的肺部X线表现分析

本文报道28例皮肌炎患者的肺部X线表现,其中25例(89.3%)有异常。作者认为本病患者大多有肺部损害,但范围和程度各不相同。     

48例系统性硬皮病的心脏损害分析

回顾性分析48例系统性硬皮病(SS)患者的临床和实验室资料.分析SS患者的心脏损害.结果:48例SS患者中,临床有心脏损害19例(39.58%),心电图、超声心动图、X线胸片检查发现心脏异常者分别19例(39.58%)、11例(22.92%)和2例(4.17%);其中以心律失常和瓣膜病变较为常见.SS心脏受累并不少见,实验室检查对SS早期心脏损害诊断有一定价值.     

儿童皮肌炎27例临床特点分析

目的探讨儿童皮肌炎的临床特点。方法对27例儿童皮肌炎的临床表现及实验室检查结果进行回顾性分析。结果本组JDM中,男女比例为1:1.7,首发症状以皮疹为主,儿童患者的面部水肿性紫红斑阳性率显著高于成人患者,发热、关节痛、心脏受累、吞咽困难的阳性率显著低于成人患者,未见间质性肺炎及恶性肿瘤。结论儿童皮肌炎以女性多见,皮肤损害为首发症状,系统损害轻,且不伴发恶性肿瘤,预后较好。     

112例皮肌炎临床分析

目的探讨皮肌炎患者的临床特点、血清学改变、实验室检查及合并恶性肿瘤的发生率。方法总结112例皮肌炎患者发病年龄、首发症状、皮疹表现、受累肌群、血清肌酶特征、伴发恶性肿瘤情况,并进行分析比较。结果①患者62.5%以皮炎为首发症状,主要以眶周融合紫红色斑为主要表现。②患者以四肢肌群受累为主。③恶性肿瘤发生率占17.86%,④乳酸脱氢酶(LDH)和磷酸肌酸激酶(CK)阳性率分别为72.32%,46.43%,与国内报道基本一致。结论特殊的皮疹表现及血清肌酶增高对皮肌炎有诊断意义,在诊疗皮肌炎患者中,应注意有无合并肿瘤,对判断预后有重要意义。     

多发性肌炎和皮肌炎70例临床分析

目的:分析多发性肌炎(PM)和皮肌炎(DM)的临床特征.方法:对70例多发性肌炎和皮肌炎患者的临床资料、实验室检查、治疗及转归进行回顾性分析.结果:本组男女之比为1:1.87,发病年龄13～81岁,平均51.52岁.58例皮肌炎患者以皮肤症状为首发症状者占62%,皮肤损害中以双上眼睑紫红斑最多,占70%.乳酸脱氢酶(LDH)和肌酸磷酸激酶(CPK)阳性率分别为74.3%和51.4%,抗Jo-1抗体阳性率为1.4%.肌活检肌炎性和肌电图肌源性改变分别为90%和56.9%.恶性肿瘤伴发率约为12.9%.结论:45岁以上的患者要注意对恶性肿瘤的筛查.     

43例皮肌炎患者皮损及肌肉直接免疫荧光检查分析

目的探讨皮肌炎的发病机理.方法采用抗人Ig(IgG、IgM、IgA和补体C3)DIF技术,检查了43例皮肌炎患者肌肉组织和其中21例皮肌炎患者皮肤损害的荧光分布.结果皮肌炎患者肌肉组织IgG、IgM、IgA和补体C3的阳性率分别为72.09%、23.26%、11.63%和51.16%,分布于肌膜;皮损区IgG、IgM、IgA和补体C3的阳性率分别为66.67%、19.05%、4.76%和61.90%,分布于基底膜.患者外周血IgG水平明显升高(70.83%).结论体液免疫亢进在皮肌炎的发病机理中起重要作用.     

Dermatomiositis paraneoplásica: estudio de 12 casos

Introduction and objectivesAdult dermatomyositis presents as a paraneoplastic syndrome in up to 25% of cases, but no clinical, histologic, or laboratory markers completely specific for paraneoplastic disease in dermatomyositis have been identified to date. Furthermore, studies on adult dermatomyositis do not usually report the frequency of cutaneous features of dermatomyositis in patients with associated cancer. Our aim was to review the characteristics of paraneoplastic dermatomyositis in patients seen at our hospital.Material and methodsWe studied 12 cases of paraneoplastic dermatomyositis and recorded patient age and sex, associated cancer, time between onset of dermatomyositis and cancer, emergent cutaneous manifestations, muscle involvement, dysphagia, lung disease, and levels of creatine phosphokinase and circulating autoantibodies.ResultsThe mean age of the patients was 61 years and the 2 most common malignancies were ovarian cancer and bladder cancer. The mean time between the diagnosis of cancer and dermatomyositis was 7 months and in most cases, the cancer was diagnosed first. Seven patients had amyopathic dermatomyositis. The most common cutaneous signs were a violaceous photodistributed rash sparing the interscapular area and a heliotrope rash, followed by Gottron papules and cuticle involvement. Superficial cutaneous necrosis was observed in 3 cases. Myositis-specific autoantibodies were not detected in any of the 6 patients who underwent this test.ConclusionsParaneoplastic dermatomyositis is often amyopathic. There are no specific cutaneous markers for malignancy in dermatomyositis. Myositis-specific antibodies are not associated with paraneoplastic dermatomyositis.     

Cutaneous changes of dermatomyositis precede muscle weakness

A retrospective review of 50 patients with dermatomyositis was performed to determine the temporal relationship between onset of muscle weakness and skin involvement. We found that cutaneous changes sometimes preceded muscle weakness more than a year before the onset of muscle weakness. These findings suggest that the characteristic dermatomyositis eruption without muscle weakness should not preclude a diagnosis of dermatomyositis, and these cases should be carefully followed.     

抗Scl-70和抗Jo-1抗体在系统性硬皮病和皮肌炎中的临床意义

为了提高对系统性硬皮病及皮肌炎的临床确诊率，通过ＥＮＡ抗原应用免疫印迹技术检测了１９例系统性硬皮病（ＳＳ）及２８例皮肌炎或多发性肌炎（ＤＭ／ＰＭ）患者抗Ｓｃｌ ７０及抗Ｊｏ １抗体，并与系统性红斑狼疮（ＳＬＥ）、混合性结缔组织病（ＭＣＴＤ）共６２例及５０例健康人作了对照研究。结果表明：７０ ＫＤ（抗Ｓｃｌ ７０）多肽抗体是系统性硬皮病的特异性标记抗体，阳性率３１．６％；５５ＫＤ（抗Ｊｏ １）多肽抗体是皮肌炎的特异性标记抗体，阳性率４６．４％。ＳＬＥ、ＭＣＴＤ及健康人全部阴性，从而有助于对系统性硬皮病及皮肌炎的鉴别诊断     

Dermatomyositis. Disease associations and an evaluation of screening investigations for malignancy

Fifty-three adult patients (19 men, 34 women) with dermatomyositis were studied. Two had dermatomyositis associated with benign disorders. Twenty-three (43%) had a malignancy; the risk of malignancy increased with age, but there was no sex difference. Seven malignancies were recurrences and 9 were diagnosed during investigation of dermatomyositis; these 16 were suspected clinically or from abnormal results of simple investigations. Extensive screening tests did not increase the number of malignancies diagnosed. In 7 patients, a diagnosis of malignancy was made more than 9 months after onset of dermatomyositis, although a relationship between malignancy and dermatomyositis was uncertain in two cases; the diagnosis of gynecological malignancy was missed in 2 patients despite appropriate investigations, 1 patient had poorly controlled dermatomyositis, and in 2 patients late diagnosis of malignancy was due to failure to reinvestigate relapse of previously stable dermatomyositis.     

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。     

Clinicopathological analysis of mechanic's hand associated with dermatomyositis

Mechanic's hand is often seen in the fingers of patients with dermatomyositis and is frequently associated with anti‐aminoacyl‐transfer RNA synthetase autoantibodies and interstitial lung disease. We analysed the clinical symptoms of 50 patients with dermatomyositis who had visited our department, 26 of whom also had mechanic's hand. A histological examination was carried out in 16 of the 26 cases, which revealed hyperkeratosis in all cases and colloid bodies in the epidermis in 15 cases. The number of cases of interstitial lung disease in patients with mechanic's hand (22/26, 85%) was significantly higher than that in those without mechanic's hand (12/24, 50%) (P < 0.05). Mechanic's hand is an important skin lesion of dermatomyositis, and increases the likelihood of interstitial lung disease     

皮肌炎患者肌肉病变的观察与评价

观察１１６例皮肌炎患者的肌肉病变，最常见的主诉是肌无力（８１．０３％）。６８例患者有吞咽困难，常规胸片检查发现４５例患者发生肺纹理增多，５５例有ＥＫＧ的异常。肌肉组织病理改变束周萎缩达３０％有诊断意义。强调联合肌浆酶和２４小时尿肌酸的测定，观察与评价内脏器官的累及对治疗和预后的重要性。     

Clinicopathological significance of cutaneous epidermal nuclear staining by direct immunofluorescence

Epidermal antinuclear antibody (ANA) staining was noted during routine direct immunofluorescence (DIF) of skin biopsies from 22 cases at St John's Dermatology Centre over a 2-year period. We have reviewed the clinical, serological and immunopathological features of these patients. They comprised 13 cases of lupus erythematosus (LE), 3 dermatomyositis, 1 morphoea, 1 systemic sclerosis, 1 CREST syndrome, 1 mixed connective tissue disorder and 1 probable cutaneous sarcoidosis. Five (38.4%) patients with LE had moderate to severe oral mucosal involvement. Epidermal nuclear staining (ENS) was seen following IgG deposition in 21 cases and IgA in only 1 case. Complement C3 staining was an additional feature in 1 patient. Circulating ANA was absent in 7 cases at the time of biopsy, confirming that this pattern of staining does not occur as a result of tissue con-lamination during processing. The presence of ENS by IMF corroborates a diagnosis of a connective tissue disorder, and our results suggest that it may also be associated with oral involvement in E.E.     

天疱疮、类天疱疮及皮肌炎合并恶性肿瘤临床分析

目的：了解天疱疮、类天疱疮及皮肌炎3种自身免疫性疾病合并恶性肿瘤的情况。方法：分析92例天疱疮患者、60例类天疱疮患者、174例皮肌炎患者的临床特点以及与肿瘤发生的相关因素。结果：肿瘤发生率在类天疱疮患者为10．00％，天疱疮患者为6．52％，皮肌炎患者为12．07％。与肿瘤发生可能相关的因素包括：天疱疮患者的病程(P=0．032，OR=0．718)，皮肌炎患者的发病年龄(P=0．012，OR=1．053)以及应用甲氨喋呤(MTX)(P=10．032，OR=0．273)。结论：天疱疮、类天疱疮与恶性肿瘤的发生无肯定的相关性，而皮肌炎与恶性肿瘤相关。     

Multiple Sclerotic Fibromas in an HIV‐Positive Patient Without Evidence of Cowden''s Disease

Dermatomyositis is an inflammatory condition of the skin and muscles, and an underlying malignancy is noted in 10% or more of cases. Clinical features of dermatomyositis include increasing general fatigue and proximal (thighs and shoulders) muscle weakness accompanied by erythematous lesions of the skin. There have been several distinct types of dermatomyositis described. Here we describe a case of vesiculo‐bullous dermatomyositis, which is a rare variant of dermatomyositis. A 49‐year‐old woman was admitted to our hospital with a painful erythematous vesicular eruption of the face, trunk and extremities. In addition, edema of the face and fever were observed. Clinically, dermatomyositis was considered because of typical skin rashes (Gottron's papules, periorbital heliotrope rash and poikiloderma) and serum creatine phosphokinase level of 1,031 IU/L. A skin biopsy was performed. Microscopically, subepidermal vesiculation with marked edema was present. Lymphoplasmacytic infiltration was also observed in the upper dermis. So far only a few case reports of vesiculo‐bullous dermatomyositis have been reported in the literature. It should be kept in mind that dermatomyositis may present subepidermal vesiculation in order to avoid a misdiagnosis and unnecessary delayed treatment. Furthermore, an internal malignancy should be considered in such a variant of dermatomyositis.