20例皮肌炎的系统病变和并发症分析

临床资料　男8例,女12例,发病年龄10～60岁,病程2月～3年。诊断标准:按日本厚生省《特定疾病研究班1980年的皮肌炎诊断指南》诊断。20例中皮肌炎19例,多发性肌炎1例。根据Bohan的分型,本组型多发性肌炎1例(5%),型皮肌炎8例(40%),型伴恶性肿瘤皮肌炎1例(5%),型儿童皮肌炎1例(5%),型伴重叠综合症皮肌炎3例(15%),伴系统性病变6例(30%)。伴发系统病变:食管受累及吞咽困难4例,急性黄疸性肝炎、间质性肺炎、雷诺征、钙质沉着各2例,心肌病变、肠麻痹、胃癌、乳腺瘤、淋巴结增生各1例20例皮肌炎的系统病变和并发症分析@殷秀琴$齐齐哈尔医学院第二附…     

局限性盘状红斑狼疮的皮肤钙质沉着症

报告一例长期患有 DLE 妇女的皮损中的皮肤钙质沉着症,钙质沉积在真皮中层和深层,周围绕以阿尔森兰液染色阳性的均匀胶原。皮肤钙质沉着症在硬皮病或皮肌炎的病人中较常出现。但在红斑狼疮中则十分罕见。就我们所知,这是 DLE 的第一例皮肤钙质沉着的报告。皮肤钙质沉着症常报告并发于儿童的进行性系统性硬皮病和皮肌炎中,但罕见于红斑狼疮,1961年 Keats 描述了 SLE 的皮肤钙化,以后,又报告了十三例,我们所报告的似     

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。     

内分泌、代谢、营养障碍性皮肤病

20070757 皮肤淀粉样变性皮损中T／B细胞的检测；20070758 维A酸类药物联合苦参素治疗原发性皮肤淀粉样变病22例临床观察；20070759 痒疹样结节型皮肤淀粉样变性1例；20070760 伴抗基膜自身抗体的皮肤异色病样淀粉样变病；20070761 特发性皮肤钙质沉着症1例[编按]     

皮肌炎55例回顾性临床分析

目的:探讨皮肌炎的临床特征和治疗方法。方法:对55例皮肌炎住院患者进行回顾性分析。结果:55例皮肌炎患者中女:男为2.2:1,儿童5例,40岁以上42例。成人患者中10%合并恶性肿瘤。92.1%有双眼睑紫红色水肿性斑片,58.2%有Gottron丘疹,98.2%感乏力,75.6%有肌痛和肌压痛。76.9%血清LDH升高,61.1%CK升高,39.1%基底膜带免疫球蛋白沉积。糖皮质激素联合MTX治疗20～40d病情控制。结论:40岁以上患者易合并肿瘤,肌酶中LDH敏感性最高,CK可以作为糖皮质激素减量的参考指标。糖皮质激素联合免疫抑制剂是目前治疗皮肌炎的主要方法。     

皮肤钙质沉着症及治疗

目的:探讨皮肤钙质沉着症的临床特点及治疗方法.方法:回顾性分析2例皮肤钙质沉着症的临床资料.结果:1例头皮皮肤钙质沉着症为外伤性皮肤钙质沉着症,经皮肤扩张及皮瓣转移修复的手术方法治愈;另1 例为特发性阴囊皮肤钙质沉着症,因局部阴囊皮肤修复难度较大而放弃手术治疗.结论:皮肤钙质沉着症为临床少见病,组织病理活检可确诊,手术治疗是有效可靠的治疗方法,切除范围要足够以免复发,一期皮肤扩张器埋植,二期皮瓣转移修复对头皮病灶效果较满意.     

幼年皮肌炎的临床表现及免疫学特点

本文显示两性的幼年皮肌炎发病率是相等的，并多伴有瘙痒，在肌肉及皮下组织中有钙质沉着是其特征之一，其心脏及呼吸系统症状的发生率也低于成年皮肌炎，不伴发恶性肿瘤是其另一个特点。     

Wong型无肌病性皮肌炎一例

患者,女,72岁。头面颈部红斑、丘疹伴瘙痒16年,日晒后加重。实验室检查示：抗TIF1-γ抗体阳性。额部皮肤组织病理表现为表皮角化过度,局灶性角化不全和界面皮炎改变。结合患者典型皮肌炎皮肤表现和额部毛发红糠疹样皮疹,诊断为Wong型无肌病性皮肌炎。给予阿维A、海棠合剂、1%吡美莫司乳膏等治疗,病情逐渐好转。     

皮肌炎83例临床分析

回顾性分析我院2003-2007年明确诊断的83例皮肌炎（DM）患者,针对性别、年龄、职业、诱因、病程、临床表现、实验室检查、伴发疾病、死亡原因等参数做分析.比较活动性间质肺病组、非活动组及无肺病组的病死率. 皮肌炎常见于女性（74.7%）,发病年龄集中在40～60岁.大部分患者无明显诱因,部分病例显示感染（8例）、劳累（5例）、染发（5例）及紫外线（1例）是诱因.从发病到就诊平均时间为半年左右.95.1%有颜面红斑,42.7%有Gottron征,41.5%有吞咽困难,39例做了肌肉活检,其中37例活检阳性,阳性率94.9%.82例作了肌电图,72例显示肌源性损害,阳性率为87.8%,肌电图93.2%有三角肌损害.22例有肺间质病变,15例合并肿瘤,其中80.0%伴肿瘤的皮肌炎患者未见肺间质病变.有5例死亡,4例死于呼吸衰竭,1例死于肿瘤.伴活动性肺间质病变组病死率为25.0%,明显高于非活动性肺间质病变组（0）和无肺间质病变组（0,P＆lt;0.05）.皮肌炎临床表现具有多样化和复杂化的特点,肌肉活检、肌电图、肌酶是皮肌炎重要的诊断依据.皮肌炎常伴发肿瘤和间质性肺炎,肿瘤和活动性肺间质病变是相互独立的预后不良因素.     

皮肌炎83例临床分析

回顾性分析我院2003-2007年明确诊断的83例皮肌炎(DM)患者,针对性别、年龄、职业、诱因、病程、临床表现、实验室检查、伴发疾病、死亡原因等参数做分析.比较活动性间质肺病组、非活动组及无肺病组的病死率. 皮肌炎常见于女性(74.7%),发病年龄集中在40～60岁.大部分患者无明显诱因,部分病例显示感染(8例)、劳累(5例)、染发(5例)及紫外线(1例)是诱因.从发病到就诊平均时间为半年左右.95.1%有颜面红斑,42.7%有Gottron征,41.5%有吞咽困难,39例做了肌肉活检,其中37例活检阳性,阳性率94.9%.82例作了肌电图,72例显示肌源性损害,阳性率为87.8%,肌电图93.2%有三角肌损害.22例有肺间质病变,15例合并肿瘤,其中80.0%伴肿瘤的皮肌炎患者未见肺间质病变.有5例死亡,4例死于呼吸衰竭,1例死于肿瘤.伴活动性肺间质病变组病死率为25.0%,明显高于非活动性肺间质病变组(0)和无肺间质病变组(0,P<0.05).皮肌炎临床表现具有多样化和复杂化的特点,肌肉活检、肌电图、肌酶是皮肌炎重要的诊断依据.皮肌炎常伴发肿瘤和间质性肺炎,肿瘤和活动性肺间质病变是相互独立的预后不良因素.     

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.     

Calcinosis cutis in autoimmune connective tissue diseases

Calcinosis cutis is a chronic condition involving insoluble calcified deposits of the skin and subcutaneous tissue. It is commonly associated with autoimmune connective tissue diseases and can be a source of pain and functional disability. The likelihood of developing calcinosis varies among the autoimmune connective tissue diseases, with systemic sclerosis and dermatomyositis being the most commonly associated. Identification of therapy for this challenging disorder has been hampered by a paucity of large controlled trials. Although there is no uniformly effective treatment for calcinosis cutis, several surgical and medical therapies have demonstrated varying degrees of benefit in the treatment of calcinosis, including surgical excision, laser therapy, extracorporeal shock wave lithotripsy, diltiazem, minocycline, colchicine, and topical sodium thiosulfate, along with others. Recommendations for the diagnosis and therapy of calcinosis cutis in patients with autoimmune connective tissue diseases are discussed.     

Subepidermal Calcified Nodule: Report of Two Cases and Review of the Literature

Subepidermal calcified nodule (SCN) is a form of calcinosis cutis that most commonly occurs in children. The typical clinical presentation is of a solitary verrucous nodule on the face, but occasionally multiple lesions may be seen. We describe SCN on the eyelids of two female patients; one of whom had four lesions. The histopathology was typical of calcinosis cutis. Both patients were in good health.     

Pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis

A case of pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis (DM) is described. The patient was a 38-year-old woman with a history of dermatomyositis for 3 months. Yellowish, hard, papulo-plaque lesions, which looked like those of pseudoxanthoma elasticum, were noted on her left axilla. Calcium deposition was confirmed by X-ray, histopathological, and electron microscopic examinations. Histopathological and histochemical examinations showed acicular calcium deposition in the middle and deep dermis surrounded by mucin. Electron microscopic examination revealed that the calcium deposition was not on collagen fibers. These morphological features were distinct from those of PXE. We proposed the possibility that degenerated mucin or degenerated elastic fiber might result in subsequent calcium deposition in reticular calcinosis in adult DM. The calcification clinically disappeared without any specific treatment except for prednisolone and cyclophosphamide.     

Regression of cutis calcinosis with diltiazem in adult dermatomyositis

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.     

The phenotypic profile of dermatomyositis and lupus erythematosus: a comparative analysis

Background: The mechanisms which regulate cutaneous inflammation in the setting of collagen vascular disease have been a topic of recent interest; emphasis has been placed on type I interferon‐associated recruitment of CXCR3+ lymphocytes in dermatomyositis (DM). Methods: On a total of 42 biopsies from patients with DM, systemic lupus erythematosus (SLE), discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE) comprehensive phenotypic studies were performed to explore the practical value of phenotypic analysis in the subclassification of lesions of collagen vascular disease. Results: The infiltrate in DM was of mild intensity compared to lupus erythematosus (LE). The dominant mononuclear cell in DM exhibited a CD4/CXCR3‐positive phenotype while biopsies of SLE typically showed a dearth of CXCR3‐positive cells. CD8 and CD20 lymphocytes were greatest in SLE and DLE, respectively. CD123 plasmacytoid dendritic cells, seen in most cases, were most frequent in cases of SCLE; CD83 expression was minimal. Endothelial MXA expression was a characteristic feature of DM. CD123 and MXA expression within inflammatory cells and keratinocytes was most conspicuous in areas of interface injury. Cutaneous lymphocyte antigen (CLA) expression was diminished in the dermal infiltrate in most cases of DM and LE. T regulatory cells never exceeded 15% of the infiltrate and were the least in the setting of DM and LE. Conclusions: An interferon‐α‐inducible cytokine milieu is common in SLE, DLE, SCLE and DM. In addition, there are phenotypic differences as alluded to above that may be of some practical value in separating these distinctive subsets. Features not previously emphasized such as MXA endothelial cell staining in DM and the lack of staining for CD83 and CLA in lesions of collagen vascular disease may be of diagnostic value. Magro CM, Segal JP, Crowson AN, Chadwick P. The phenotypic profile of dermatomyositis and lupus erythematosus: a comparative analysis.     

Lymphoma Arising From a Calcinotic Lesion in a Patient with Juvenile Dermatomyositis

Abstract:  Approximately one-third of adult patients with dermatomyositis develop malignancy, usually within 1 year of diagnosis of dermatomyositis. This phenomenon is not seen in patients with juvenile dermatomyositis. We are reporting a case of B-cell lymphoma arising from a calcinotic lesion in a 14-year-old girl who had been diagnosed with juvenile dermatomyositis 3 years prior. Our review of the literature revealed only one other case of malignancy arising from an area of calcinosis.     

Calcinosis Cutis in Juvenile Dermatomyositis Responsive to Aluminum Hydroxide Treatment

Calcinosis cutis is a frequent complication of juvenile dermatomyositis; however, its treatment remains unsatisfactory. We report a case of calcinosis cutis in juvenile dermatomyositis which was successfully treated with oral aluminum hydroxide. Almost complete clearing of calcinosis was observed after 8 months of therapy. There were no adverse effects from therapy. Aluminum hydroxide is worth trying in treating calcinosis cutis.     

Linear morphea with overlying lichen sclerosus and calcinosis cutis associated with juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is associated with many distinguishing features including cutaneous calcinosis, vasculitis, and ulcerated lesions. In this case, we describe an unusual presentation in a 12-year-old girl who had muscle weakness along with linear morphea over the right upper and lower extremities with overlying lichen sclerosus and calcinosis cutis. Of interest, these initial cutaneous manifestations occurred years before onset of myositis.