213例皮肌炎临床和血清肌酶特征分析

目的：探讨皮肤科门诊定期就诊的皮肌炎患者临床和血清肌酶特征及长期皮质类固醇治疗引起的副作用。方法：分析213例皮肌炎的首发症状及病程中临床表现和血清肌酶分析情况，并按年龄、性别组进行分析比较。结果：所有患者一般特征、临床表现和血清肌酶与国内外报道基本一致，首发症状为肌无力者占96．24％，成年组伴发恶性肿瘤者为23．94％。结论：儿童皮肌炎预后较好，而成人皮肌炎伴发肿瘤和肺部受累提示预后较差，皮质类固醇副作用以合并浅表真菌感染最常见。     

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.     

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。     

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     

儿童皮肌炎与成人皮肌炎患者临床和实验室资料对比分析

为了探讨儿童皮肌炎（JDMS）与成人皮肌炎（ADMS）临床表现上的差异，将35例JDMS和139例同期住院的ADMS患者的临床及实验室资料进行对照研究，结果显示JDMS男女比为1：1．3，而成人男女比为1：2．1，JDMS眼周紫红斑，Gottron's丘疹的高发生率及ADMS相似，但肌炎症状较轻，合并症较少，肌萎缩，吞咽困难，间质性肺炎，恶性肿瘤的发生率均明显低于成人患者（P＜0．05，P＜0．01，P＜0．01，P＜0．05），血清CPK，IgG升高率亦明显低于成人患者（P＜05，P＜0．01），而发热，上呼吸道感染的发生及抗“O”阳性率则明显高于成人患者（P＜0．01，P＜0．05，P＜0．01），结果提示JDMS与ADMS在发病机理及临床特点上均有差异，皮损特征对儿童皮肌炎的早期诊断更为重要。     

儿童系统性红斑狼疮18例分析

目的：了解儿童系统性红斑狼疮的临床及实验室检查特征。方法：对18例儿童系统性红斑狼疮与54例成年人患者的临床特点进行对照研究。分析其首发症状,临床表现及实验改变。结果：儿童患者肾脏损害的严重程度比成人重,其它器官损害也多于成人,但无统计学意义,未成年人组C3下降程度明显高于成年组,而CH50降低的发生率明显高于成年组,结论：系统性红斑狼疮儿童患者临床及实验室检查与成人相比存在某些程度差异。     

皮肌炎合并恶性肿瘤82例临床分析

目的了解皮肌炎合并恶性肿瘤的几率和临床特点。方法回顾1991年1月—2011年2月中山大学附属孙逸仙纪念医院住院治疗的511例皮肌炎患者,统计和分析其合并恶性肿瘤情况。结果皮肌炎合并恶性肿瘤82例,占皮肌炎患者16.05%。以41~60岁年龄段发生率高。鼻咽癌最常见,共48例,占恶性肿瘤58.54%。肺癌11例,卵巢癌6例。先发皮肌炎后发恶性肿瘤者64例;皮肌炎与恶性肿瘤同时发生11例;先发恶性肿瘤后出现皮肌炎者7例。治疗恶性肿瘤后皮肌炎症状好转,恶性肿瘤恶化皮肌炎症状加重。结论皮肌炎合并恶性肿瘤常见于年龄40岁以上患者,广东地区皮肌炎合并的恶性肿瘤以鼻咽癌最常见,其次是肺癌和卵巢癌。     

儿童皮肌炎25例临床分析

皮肌炎(DM)是一种以进行性对称性近端肌无力和典型的皮肤损害为特征的自身免疫性疾病。尽管儿童皮肌炎的预后较成人皮肌炎好，但仍有死亡和复发。为了进一步探讨儿童皮肌炎的临床特征和影响预后因素，现对我院25例儿童皮肌炎住院患儿的临床资料分析如下。     

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。     

青少年与成人皮肌炎/多发性肌炎的比较分析

目的：探讨青少年皮肌炎/多发性肌炎（JDM/PM）的临床表现和预后特点。方法：比较分析25例JDM/PM与143例成人皮肌炎/多发性肌炎（DM/PM）的临床表现、辅助检查结果及预后情况。结果：与成人DM/PM相比,JDM/PM男多于女;初发症状中肌痛和体重下降较少见,临床表现中光敏较多见,较少出现肌痛、咽喉部肌群受累、消瘦和肺、胸膜受累;未见并发恶性肿瘤和死亡;血、尿常规,免疫学,血清酶,肌电图及肌活检结果等则与成人无统计学差异。结论：JDM/PM具有一定的临床表现特征,肺、胸膜较少累及,预后较好。     

39例成人与16例儿童皮肌炎比较分析

目的：比较成人与儿童皮肌炎临床与实验室检查上的差异,指导临床实践。方法：回顾性总结和分析资料完整的皮肌炎患的临床表现及实验室检查结果,采用精确概率法统计分析两组患之间的差异。结果：在儿童组中女性发病明显高于男性,男：女为1：7,在临床表现上并发疾病及合并肿瘤的发生率成人组高于儿童组;儿童组表现发热、肌压痛、甲周红斑和／或毛细血管扩张（X^2＝11．75,P＜0．05）的发生率高于成人组;实验室检查上LDH、CPK、AST在儿童的敏感性高于成人;成人组ESR值升高发生率高于儿童组,心肌受损和肺部感染发生率高于儿童组。结论：对儿童皮肌炎的皮损除检查眼睑外,应仔细检查甲周,对成人皮肌炎应注意排除存在恶性肿瘤的可能和注意心肌受损及肺部感染。     

Infrapatellar hypertrichosis: an unusual cutaneous manifestation of juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is an uncommon inflammatory myopathy with characteristic cutaneous manifestations. Certain clinical features such as cutaneous calcification, lipoatrophy, and generalized hypertrichosis can occur in JDM but are uncommon in the adult form of the disease. We report a young girl who presented with dramatic infrapatellar hypertrichosis along with more classic clinical signs of JDM, including heliotrope periorbital eruption, photosensitivity, and profound proximal muscle weakness. The clinical course was favorable, with improvement of cutaneous and muscular abnormalities after corticosteroid treatment.     

儿童白癜风396例临床分析

目的 探讨汉族儿童白癜风的临床特点。方法 采用问卷调查方式对浙江省396例儿童白癜风及825例成人白癜风患者进行临床分析，用SPSS 10.0软件包对资料进行分析。结果 396例儿童白癜风患者中男183例占46.2%，女213例占53.8%，与成年患者相比，男女之间无性别差异。儿童患者中节段型的比例为33.3%，明显高于成人患者的13.5%，泛发型比例为4.3%，显著低于成人患者的11.6%。儿童患者家族史阳性比例为12.4%，成人患者家族史阳性比例为12.7%，两组间差异无统计学意义。有家族史患者一级亲属的发病率均明显高于二级亲属。儿童患者的诱发因素以外伤为主，伴发晕痣的比例明显高于成人患者；成人患者伴发甲状腺疾病的比例显著高于儿童患者。结论 儿童白癜风临床特点与成人相比有一定差异。     

Characteristics of vitiligo in Korean children

Eighty Korean children (ages 8 months-12 years) with clinical and/or histopathologic diagnoses of vitiligo were evaluated; 39 boys and 41 girls. The mean age at first visit was 7.9 years and the mean age at onset was 5.6 years. The most common site of onset was the head/neck area (58.8%), followed by the trunk and lower limbs. The children were compared with a control group of 422 adults with vitiligo. Children comprised 16% of the total vitiligo patients and adults comprised 84%. A family history of vitiligo was found in 11 (13.8%) children, compared to 10.7% in the adult group; poliosis in 20 (25. 0%); halo nevi in 2 (2.5%), compared to 4.0% in the adult group; combined autoimmune and endocrine diseases in 1 (1.3%), compared to 7.6% in the adult group; and segmental vitiligo in 26 (32.5%), compared to 13.0% in the adult group. The combined diseases were significantly less often found in children than adults (p < 0.01), and segmental vitiligo was significantly more often associated with children (p < 0.0001). Our study did not show a higher prevalence of vitiligo in girls as reported in other studies, which may indicate racial differences. Of the total 502 patients, only 1 patient with segmental vitiligo had halo nevi. Sixty-four percent of the children with vitiligo responded to treatment, compared to 57% of the adults.     

Clinical comparison of psoriasis in Korean adults and children: correlation with serum anti-streptolysin O titers

Psoriasis is a relatively common disorder in children and can be triggered by an upper respiratory tract infection. The aim of this study was to compare the clinical features of psoriasis in children and adult. In addition, we evaluated the relationship between anti-streptolysin O (ASO) titers and the clinical features of psoriasis. A total of 30 childhood psoriasis patients and 30 adult psoriasis patients were evaluated. Childhood psoriasis had a facial predominance when compared with the adult psoriasis. The childhood psoriasis patients with high ASO titers had guttate psoriasis more frequently than patients with normal ASO titers. In children with plaque-type psoriasis, psoriasis area and severity index score was increased in the high ASO titer group than normal ASO titer group. In conclusion, if the children with psoriasis show increased ASO titer, the physician should pay attention to the worsening of the psoriasis. Furthermore, early treatment of streptococcal infections might be beneficial in childhood psoriasis.     

Juvenile dermatomyositis: immunopathogenesis, role of myositis-specific autoantibodies, and review of rituximab use

Juvenile dermatomyositis (JDM) is an autoimmune disease of the skin and muscle that affects children. The etiology is poorly understood, but genetic susceptibility, environmental triggers, and abnormal immune responses are each thought to play a part. T cells have traditionally been implicated in the immunopathogenesis of JDM, but dendritic cells, B cells, and microchimerism are increasingly associated. Additionally, myositis-specific autoantibodies (MSA) can be present in the sera of affected patients and may correlate with distinct clinical phenotypes. Given the role of humoral immunity and MSA, there has been recent interest in the use of rituximab to treat JDM. Early results are mixed, but it is hoped that a prospective clinical trial will shed light on the issue in the near future.     

儿童多发性肌炎与皮肌炎17例临床分析

目的探讨儿童皮肌炎/多发性肌炎(JDM/PM)的临床特点、辅助检查与治疗。方法回顾性分析本院1994年1月~2004年12月诊治的17例JDM/JPM。结果主要有肌无力、肌痛或肌压痛、关节疼痛和发热等;存在肺、心脏、胃肠道、肾损害,未见伴恶性肿瘤和钙质沉着者;LDH、HBDH、AST、CPK、α2、γ球蛋白升高,HB降低,ESR增快,RF阳性;EMG示肌源性改变,肌活检异常。结论本病以肌肉症状最多见,肌酶升高显著,以心、肺损害多见,经激素治疗均有所缓解,预后较好。     

Juvenile dermatomyositis--statistical observation of 105 patients with dermatomyositis

Statistical observation about the clinical items of 105 patients with dermatomyositis (DMS) attending Department of Dermatology, Nagoya University Hospital and some hospitals in Aichi Prefecture, during the year of 1965 to 1989, was carried out. Clinical features of the patients with juvenile DMS obtained from the observation were as follows. 1) The male:female ratio was 1.3:1 in juvenile DMS. The evident difference such as the predominance of females in adult DMS was not found. 2) The tendency that cutaneous manifestations usually preceded muscular manifestations was observed, and the muscular manifestations except severe symptoms were seen with a high incidence throughout the entire clinical course. 3) In the laboratory examinations, the incidence of elevation of serum aldolase concentrations in children was significantly higher than that in adults (p less than 0.05). Serum aldolase concentrations were usually elevated at onset or prior to the onset of muscular manifestations. Therefore the measurement of serum aldolase levels was considered to be useful for early diagnosis of juvenile DMS. The positive rate of antinuclear antibody in children was significantly lower than that in adults (p less than 0.001). 4) None of the children had any complications such as malignant tumors, interstitial pneumonia and pulmonary fibrosis, and none of them died. With regard to the outcome, the incidence of "remission or improvement" in children was significantly higher than that in adults (p less than 0.05). In the group of "same or worse", the children mainly had the cutaneous manifestations which were difficult to treat, compared with the adults. These results suggest that juvenile DMS may be a different disorder, probably a syndrome, from adult DMS.