Pneumomediastinum and Subcutaneous Emphysema Associated with Fatal Interstitial Pneumonia in Dermatomyositis

We described a 65-year-old woman who died of acute interstitial pneumonia associated with dermatomyositis. Subcutaneous emphysema and pneumomediastinum simultaneously developed. The association of the pulmonary rupture with vasculitis has been assumed as the common cause in interstitial pneumonia. Diffuse alveolar damage, however, might have led to the pneumomediastinum and subcutaneous emphysema in our patient, who had no signs of cutaneous vasculitis.     

Dermatomyositis with Multiple Skin Ulcers Showing Vasculitis and Membrano-cystic Lesion

A 63-year-old woman with dermatomyositis (DM) developed multiple punched-out ulcers of the skin. She had not only a past history of gastric cancer 10 years prior to the onset of DM, but also a familial history of cancer. A skin biopsy from one of the ulcers revealed both vasculitis and membrano-cystic lesion in the same specimen. She died of interstitial pneumonitis one year after the onset of DM. Vasculitis and membrano-cystic lesion may be an important marker of a poor prognosis in DM.     

A case of dermatomyositis associated with mechanic's hand

A 67-year-old man was referred to the Department of Internal Medicine at Tokyo Medical University with interstitial pneumonia in July 1999. He presented with keratotic plaques on both palsm and on the ventral and lateral sides of his fingers. Erythematous keratosis was observed on the dosal aspect of his fingers and metatarsophalangeal (MP) joints. Edematous erythema was seen on the patient's chest, back, and the extensor surfaces of his arms. Electromyography revealed a myogenic pattern and an increased level of myogenic enzymes was found in the blood. Histological findings of the ventral sides of his fingers showed hyperkeratosis and parakeratosis of the dermal tissue and liquefaction degeneration of the basal layer at the papilla. Based on these findings, the patient was given a diagnosis of dermatomyositis associated with mechanic's hand. A systemic examination confirmed interstitial pneumonia and carcinoma of the duodenal papilla. Mechanic's hand is a type of dermatitis associated with myopathy first reported by Stahl et al. in patients with collagen disease. We report herein the first documented case of mechanic's hand in Asians.     

Successful Treatment of a Therapy-Resistant Severely Pruritic Skin Eruption of Malignancy-Associated Dermatomyositis with High-Dose Intravenous Immunoglobulin

Malignancy-associated dermatomyositis developing in a middle-aged Japanese female was successfully treated by removal of her gastric cancer. However, five years later, concomitant with catching a cold, her severely pruritic skin lesions recurred on exposed areas. Six years after the start of corticosteroid therapy for her annoying skin lesion, we started to treat her with high-dose intravenous immunoglobulin (IVIG) infusion therapy because of the difficulty of reducing the dosage of oral prednisone, that had secondarily induced adverse effects. After five courses of the therapy, her recalcitrant, pruritic, erythematous skin lesions improved dramatically, enabling a satisfactory reduction in the dosage of oral prednisone. There were no significant adverse side effects with IVIG.     

Three cases of 'mechanic's hands' associated with interstitial pneumonia: possible involvement with foot lesions

We describe three cases of the rare combination of lateral erythema and hyperkeratosis of the fingers that typify a condition known as 'Mechanic's Hands'. The first and the third cases were unusual in that the condition was associated with cutaneous involvement of the feet and interstitial pneumonia but not with myositis, or with only mild muscular involvement, while the second case was typical, being accompanied by myositis and detectable antibodies against histidyl transferase. We propose that Mechanic's Hands can occur in association with foot lesions and interstitial pneumonia, even if it is not accompanied by myositis.     

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。     

An Autopsy Case of Malignant Lymphoma with Lyell's Syndrome

We present a case of fatal Lyell's syndrome which developed following a CT examination using omnipaque 3000 contrast medium. A 59-year-old man was suffering from malignant lymphoma. He was readmitted to this hospital due to relapse of fever and lymph node swelling. On the day of readmission, generalized erythema, purpura, and mucosal erosions developed after a CT examination. Steroids and chemotherapy were ineffective, and he expired approximately two weeks after admission. Drug-induced dermatopathy or leukemic cell infiltration in the skin was clinically suspected. Histological findings disclosed toxic epidermal necrolysis.     

皮肌炎并发间质性肺病32例分析

目的 探讨皮肌炎(DM)并发间质性肺病(ILD)的临床特点和意义。方法 收集北京协和医院1970-1997年门诊和住院长期随诊的216例DM患者,符合Bohan和Peter1975年诊断标准。随诊2~27年,平均(5.8±4.7)年。DM合并ILD32例,DM无ILD184例。比较两组临床资料和实验室各指标,以这些参数作统计学分析。结果 DM患者中,ILD组初发症状中发热(28.2%)显著多于无ILD组(6.5%),P<0.01。病程中ILD组的发热、关节炎、皮肤溃疡和类风湿因子阳性明显增高,分别为71.9%、62.5%、31.3%和24.1%,无ILD组分别为34.2%、8.7%、8.1%,两组比较,P均<0.01或<0.05。而两组患者眼睑紫红斑、Gottron征、吞咽困难、肌肉痛、肌无力、抗核抗体阳性和肌电图肌源性改变发生率差异均无显著性(P均>0.05)。ILD组32例中死亡13例(40.6%),病情改善15例(46.9%),缓解而停用皮质类固醇4例(12.5%)。184例DM无ILD者死亡30例(16.3%),两组死亡率差异有显著性(P<0.01)。结论 DM患者有ILD者发热、关节炎、皮肤溃疡和类风湿因子阳性明显增多。这组患者治疗困难,死亡率高。     

马尔尼菲青霉病致溶骨性损害2例

例1患者女,54岁,反复咳嗽、咳痰11月,再发加重1月入院,住院期间患者全身皮肤突然出现多个紫红色大小不等的结节,发热,因外力致左肩部疼痛,活动受限;例2患者男,43岁,因反复发热、颈部肿物1年,右上臂疼痛13天入院,因外力致右上臂活动受限;以上2例经组织及脓液培养均确诊为马尔尼菲青霉病,X线片提示2例骨折均为病理性骨折。     

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     

Survey of the prognosis for dermatomyositis, with special reference to its association with malignancy and pulmonary fibrosis

We sent questionnaires to the major dermatological institutions in Japan concerning the course and associations of dermatomyositis (DM). Of 637 cases observed between 1973 and 1983, 68 were children. The mortality rate was 2.9% in children, 47.1% in adult males, and 30.0% in adult females. Associations with malignancies were noted in 30.1% of adults; 112 cases (19.7% of adults) died from these, but surgery improved the signs of DM in 22 cases. The main neoplasms were cancer of the stomach, lung, colon, and breast. Association with pulmonary fibrosis (PF) was observed in 85 cases (14.9% of adults) and, in spite of intensive treatment, 38 cases (6.7%) died from it. In the majority of PF cases who died within 2 months, pulmonary symptoms started within 6 months of the onset of DM. All cases of PF which occurred before the onset of DM survived. The concurrence of PF and malignancies was exceptional. Death from myocardial disease occurred in only 3 cases.     

A Case of Erythrodermic Dermatomyositis Associated with Gastric Cancer

Erythroderma is an unusual cutaneous finding associated with dermatomyositis. There are only five cases of erythrodermic dermatomyositis reported in the English literature. We treated a case of erythrodermic dermatomyositis associated with a Bormann type 1 gastric cancer. The patient had a generalized, erythematous scaly eruption consistent with erythroderma and Gottron''s papules as well as a heliotrope rash; these are the hallmark skin manifestations of dermatomyositis.     

抗MDA5抗体阳性重症皮肌炎1例

患者女,31岁,多发肌肉疼痛4个月,咳嗽、气短2个月,发热3 d.肌电图提示肌炎活动期;特发性炎性肌炎谱示:抗MDA5抗体IgG(3+),抗Ro-52抗体IgG(3+);胸部CT提示两肺间质性改变及感染.诊断:皮肌炎,肺间质病变,I型呼吸衰竭,肺部感染.     

Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

Systemic sclerosis (SSc) is a chronic systemic disease of unknown etiology characterized by vasculopathy, excessive accumulation of extracellular matrix, and fibrosis of the skin and other internal organs. Although its etiology remains elusive, approximately one third of SSc patients presents with additional autoimmune disease, which suggests that an autoimmune mechanism is a major component of the underlying pathophysiology. On the other hand, primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) are two main autoimmune liver diseases. A 41-year-old female previously diagnosed with PBC/AIH overlap syndrome presented with multiple, painful brownish to erythematous firm patches on the hands, arms, axillae, neck, abdomen, and thighs. Laboratory work-up yielded positive results for anti-nuclear antibody, anti-Ro/Sjögren''s-syndrome-related antigen A autoantibodies, and perinuclear anti-neutrophil cytoplasmic antibodies while punch biopsy of her left hand showed characteristics that are consistent with scleroderma. Herein, we report the first case of a patient with diffuse cutaneous SSc and concurrent PBC/AIH overlap syndrome and suggest that this coexistence of multiple autoimmune diseases is not a coincidence but rather that a common autoimmune pathogenesis may exist.     

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。     

皮肌炎并发卵巢癌1例及文献复习

患者女,58岁。全身起疹伴瘙痒1个月。面部、双上肢及大腿外侧出现红斑和水疱,并逐渐出现肌无力和吞咽困难。诊断:皮肌炎;卵巢癌。予激素治疗及手术切除卵巢癌组织,疗效较好。本文就本例临床特点进行总结并在发病特点、皮疹表现、血清指标和预后的因素等方面进行了文献复习。     

A Case of Calcinosis Universalis Successfully Treated with Low-Dose Warfarin

A 27-year-old male patient with calcinosis universalis resulting from dermatomyositis was successfully treated with low-dose warfarin. On his trunk and extremities, there were many subcutaneous calcified nodules, and knee flexion was difficult. After oral warfarin therapy for three years, the calcified nodules became smaller, and the knee mobility improved. His serum vitamin K level was abnormally high, decreased just after starting warfarin therapy, and then remained within the normal range. Since vitamin K has been known to play an important role in the Ca²⁺ binding process in bones or tissues, we suggest that this therapy is effective in reducing subcutaneous calcification through the vitamin K cycle.     

An extremely severe case of cutaneous calcinosis with juvenile dermatomyositis, and successful treatment with diltiazem

A case of cutaneous calcinosis associated with juvenile dermatomyositis is described. The patient was a 3-year-old girl who had been diagnosed as having dermatomyositis at age 1 year. She was treated with prednisolone, but developed multiple calcified nodules in the subcutaneous tissues and intermuscular fascia. These nodules gradually increased in size despite continual therapy with steroids and aluminium hydroxide. Treatment with diltiazem completely suppressed the development of calcinosis.     

Long‐term outcome of patients with polymyositis/ dermatomyositis and anti‐PM‐Scl antibody

Background To date, no series has analysed long‐term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti‐PM‐Scl antibody. Objectives The aims of the present study were: (i) to assess clinical features and long‐term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti‐PM‐Scl antibody; and (ii) to evaluate prevalence, characteristics and long‐term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti‐PM‐Scl antibody. Methods The medical records of 20 consecutive patients with isolated PM/DM with anti‐PM‐Scl antibody were reviewed. Results Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short‐term recurrences (during tapering of therapy) occurred in nine patients and long‐term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti‐PM‐Scl antibody exhibited severe complications, as follows: oesophageal involvement (n = 4) requiring enteral feeding in three cases, ventilatory insufficiency (n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti‐PM‐Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms (n = 2; 10%), Raynaud’s phenomenon (n = 8; 40%), arthralgia/arthritis (n = 7; 35%) and ILD (n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents. Conclusions Our series suggests that the presence of anti‐PM‐Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti‐PM‐Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti‐PM‐Scl antibody‐positive patients with PM/DM often exhibit ‘mechanic’s hands’, Raynaud’s phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA‐DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti‐PM‐Scl antibodies.     

系统性红斑狼疮伴心血管损害119例临床分析

目的　探讨系统性红斑狼疮心血管系统受损情况。方法　对 119例系统性红斑狼疮伴心血管损害的临床资料进行归纳分析。结果　心血管系统各个部位均可受累 ,以心包炎最常见 ,心脏增大次之。末梢血管病变以雷诺现象多见。结论　心电图与彩色B超检查发现心血管异常者较多见 ,除 7例外其余患者皆有多个器官受损。