皮肌炎合并甲状旁腺机能减退症1例

1 临床资料 患者女，12岁。以面部水肿性紫红色斑伴低热，乏力2个月就诊。患儿2个月前无明显诱因双手指关节伸侧出现紫红色扁平丘疹，上覆固着鳞屑，无不适，未予治疗。后渐出现甲周发红，甲护膜增厚，面颊，颈前V字区、颈后，及双眼睑水肿性红斑、乏力。     

皮肌炎合并肺大疱并发液气胸1例

皮肌炎合并肺大疱并发液气胸１例干皆诚，唐署，张定国（上海第二医科大学附属新华医院皮肤科２０００９２）患者男，３３岁，驾驶员，住院号２４７６９１。１９８８年９月，在无明显诱因情况下，面，颈部出现水肿性红斑，以上限脸为显著，且伴有全身乏力，肌肉酸痛，低热...     

皮肌炎合并白癜风1例

患者女，46岁。因面部红斑1年，四肢乏力9个月，全身泛发白斑3个月就诊。1年前无明显诱因鼻根部及双眶周围皮肤出现红斑，伴肿胀及轻微瘙痒；9个月前面部出现红斑，累及整个面部、颈项、胸骨区及前臂屈侧，同时出现四肢乏力。在外院检查示：抗核抗体(ANA)1：1000，IgA3470mg／L。肌肉活检示局灶性肌纤维颗粒变性及空泡变性，符合皮肌炎改变，给予泼尼     

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。     

皮肌炎并发鼻腔,鼻窦癌1例

皮肌炎并发鼻腔、鼻窦癌１例秦恩波（北京铁路总医院皮肤科，１０００３８）北京铁路总医院耳鼻喉科患者男，６５岁。因面部红斑、肌肉无力９个月，流涕、鼻塞６个月，症状加重１个月，于１９９１年５月７日以皮肌炎收入院。于１９９０年６月面部起红斑，不痛不痒，四肢肌...     

皮肌炎合并恶性肿瘤的临床分析

皮肌炎是一种以皮肤及横纹肌的炎症为主的全身性疾病,可合并恶性肿瘤,为探讨皮肌炎合并恶性肿瘤的临床表现。早期诊断,使本病达到及时正确的治疗,笔者收集了１９８４年９月～１９９９年８月我院皮肌炎患者的病历资料,现将其合并恶性肿瘤１８例分析报道如下：１临床资料１．１一般资料１９８４年９月～１９９９年８月,我院共收治８２例皮肌炎患者,其中合并恶性肿瘤１８例,占２１．９５％,男６例,女１２例,男：女＝１：２,年龄３６～７６岁,平均５６．０８岁,小于４０岁２例,大于４０岁１６例。１．２合并恶性肿瘤类型肺癌４例…     

皮肌炎并发横纹肌溶解症及急性肾损伤一例

正皮肌炎是一种主要累及皮肤和横纹肌的自身免疫性结缔组织病,以红斑、水肿为皮损特点,伴有肌无力和肌肉炎症、变性的疾病,常伴有关节、心肌等多器官损害。皮肌炎本身所致的肾脏受损较少见,但皮肌炎可导致横纹肌溶解症,后者可并发急性肾损伤,肾功能衰竭一旦形成,病死率很高,预后极差。我院收治1例皮肌炎并发横纹肌溶解症及急性肾损伤的患者,现报告如下。临床资料患者,男,24岁。因全身皮损伴四肢无力10余     

皮肌炎合并多发性皮肤钙沉着症2例

例1，男，18岁，主因双上肢，下肢皮肤肿胀，疼痛伴无力5年，下肢结节1年来我院就诊。患者于5年前无明显话因出现四肢皮肤浮肿发硬，疼痛伴无力，活动受限，下蹲后起立，抬腿及上台阶均困难。曾在当地医院诊为“硬皮病”，给予泼尼松60mg/日，环磷酰胺150mg/日口服，症状逐渐     

皮肌炎合并胆囊炎1例报道

患者，女，２１岁。１９９７年因皮肌炎给予皮质类固醇治疗后病情好转，后来减量至口服泼尼松１０mg／d。２００１年７月初日光曝晒后病情加重，在当地医院口服泼尼松７０mg／d及静脉滴注氨甲喋呤针１次／周（用量不详）治疗２周，而后出现腹痛，食欲不振等症状。以皮肌炎伴腹痛待诊入院治疗。入院查：体温３６．６℃，脉搏８０次／分，呼吸２０次／分，血压１４／１１kPa。查体：形体消瘦，胃区有压痛，无反跳痛和肌紧张，四肢肌肉压痛不明显，肌力正常。皮肤科所见：眼睑、面颊部对称性水肿性紫红色斑片，表面干燥伴有糠样鳞屑，有大片色…     

皮肌炎患者合并恶性肿瘤的临床分析

皮肌炎(dermatomyositis,DM)是一种结缔组织疾病,以皮肤和肌肉的炎症为主要表现,伴发恶性肿瘤的概率高.为了探讨湖南地区皮肌炎合并肿瘤的情况,我们回顾性分析了我科1990年～1998年住院病例的临床资料,现报道如下.     

Dermatomyositis

ABSTRACT: Dermatomyositis (DM) is an acquired, inflammatory muscle disorder of unknown etiology characterized by symmetrical proximal muscle weakness, inflammatory myositis on biopsy, elevation of serum muscle enzymes, abnormal electromyogram, and a typical rash, with or without internal organ involvement. Untreated muscle disease can produce severe and long-term disabilities, and the cutaneous lesions can cause considerable discomfort from intractable pruritus. A prompt and usually aggressive approach to therapy is indicated, with each patient's management depending on the severity of the skin and muscle disease and the presence or absence of multisystem involvement. The mainstay of therapy for DM is the use of systemic corticosteroids. In those patients that do not respond to steroids or develop significant steroid-related side effects, immunosuppressive agents (methotrexate, azathioprine, cyclophosphamide, and more recently cyclosporine), or intravenous immunoglobulin (IVIG) are used. General measures include bed rest, passive range-of-motion exercises, and active physical therapy. Overall the use of corticosteroids with or without immunosuppressive agents has significantly reduced mortality and increased functional recovery. Corticosteroids may not control the rash. In these cases aminoquinolone antimalarials have been advocated, though partial improvement is more common than complete control. Emollients and photoprotection are essential. In recalcitrant cases of cutaneous DM, low-dose methotrexate and IVIG have been used with success. Finally, with the recognition of distinct subsets limited to the skin (e.g., amyopathic DM), the dermatologist is confronted with the decision for appropriate therapeutic modalities tailored to the individual patient's needs.     

Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome

Although periorbital edema is a common manifestation of dermatomyositis (DM), generalized subcutaneous edema associated with DM is extremely rare. Evans syndrome is an autoimmune disease in which an individual's antibodies attack one's own red blood cells and platelets. Evans syndrome is rarely a presenting feature of DM. DM has been rarely reported to be associated with either generalized edema or Evans syndrome. We report the case of a 52-year-old Korean woman who presented with generalized subcutaneous edema, an erythematous rash, dysphagia, and proximal muscle weakness, and subsequently developed features of Evans syndrome. Treatment with high-dose glucocorticoids and an immunosuppressive agent controlled the DM, the generalized subcutaneous edema, and the Evans syndrome.     

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.     

Dermatomyositis panniculitis: A case report

Dermatomyositis‐related panniculitis is a rare cutaneous manifestation of dermatomyositis. There are few reported cases in the medical literature. We present the case of a 60‐year‐old woman with a 2‐year history of dermatomyositis and recent biopsy‐confirmed panniculitis treated with prednisone, cyclophosphamide and i.v. immunoglobulin.     

Dermatomyositis Associated with Primary Intramuscular B Cell Lymphoma

A rare case of dermatomyositis associated with primary intramuscular malignant lymphoma is described. A 40-year-old Japanese woman noticed swelling of the right thigh during the treatment of dermatomyositis with prednisolone, azathioprine and cyclophosphamide. A biopsy specimen taken from the right quadriceps muscle revealed infiltration of lymphoma cells which were positive for CD20. We reviewed 12 cases of dermatomyositis associated with malignant lymphoma reported in Japanese literature between 1984 and 1996.     

Dermatomyositis related to autoimmune thyroiditis

Background and objective Reports on the coexistence of dermatomyositis (DM) with autoimmune thyroiditis (AIT) are very few. Our aim is to define the relationship of the two conditions, identify clinical, laboratory, electromyographic and pathologic features of coexistent DM and AIT. Methods We underwent a MEDLINE search to identify relevant literature published in the past 30 years. Concurrently, we analysed retrospectively medical records of five patients diagnosed with DM and AIT from our hospital. Results Eleven cases were included. 90.9% of patients were female with a mean (SD) age of 44.18 (13.11) years for DM at diagnosis, and 39.00 (7.81) years for AIT. AIT can precede or parallel the diagnosis of DM. The most common comorbidities included hypothyroidism (90.9%), cardiopulmonary diseases (63.7%) and overlap syndrome (27.3%), while only one case had malignancy. The most common clinical manifestations were: muscle weakness (100%), polyarthralgia (45.5%), heliotrope rash (45.5%), myalgia (36.4%), and Raynaud’s phenomenon (27.3%). The abnormalities on electromyography and muscle/skin biopsy of DM related to AIT did not differ from those findings of DM, while none of these reports were normal. All patients received both the treatment of corticosteroids and levothyroxine, and only 27.3% of patients had a good prognosis. Conclusion Prevalence of cancer in coexistent DM and AIT may be very low. Also, it is reasonable to suggest that DM patients with AIT should be routinely evaluated for thyroid function and the emergence of comorbidities. Moreover, corticosteroids combined with levothyroxine may be useful for these patients as a standard treatment.     

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。     

Analysis of Lymphocyte Subpopulations in Peripheral Blood in Adult and Juvenile Cases of Dermatomyositis

Dermatomyositis, recognized as an autoimmune disorder, occurs not only in adults but also in children. In this study, we evaluated lymphocyte subpopulations in the peripheral blood of 24 adult dermatomyositis and 14 juvenile dermatomyositis patients and in 17 healthy adults and 9 healthy children by flow cytometry using monoclonal antibodies. When compared with healthy adults and adult patients with inactive dermatomyositis, the adult patients with active disease had significantly lower percentages of CD3⁺ and CD8⁺ cells and a significantly higher percentage of CD20⁺ cells. In contrast, juvenile dermatomyositis patients had lymphocyte subpopulations not significantly different from those of healthy children; the activity/inactivity of disease did not make any difference. These results support our hypothesis that adult and juvenile dermatomyositis may be diseases of entirely different scope.     

An Autopsy Case of Dermatomyositis with Rapidly Progressive Diffuse Alveolar Damage

A 47-year-old woman visited a clinic with dyspnea which had continued for two months and was followed by general fatigue and fever. Antibiotics were not effective. Edematous erythema occurred on her face, elbows, knees and feet, and she entered our hospital. A skin biopsy revealed interface dermatitis with severe edema and mucinosis in dermis. Diffuse bilateral infiltration was observed in the chest X-ray, and laboratory findings showed increased LDH, GPT, GOT and CPK. No antinuclear factor was detected. Her respiratory condition rapidly worsened, and she died eight days after hospitalization in spite of corticosteroid pulse therapy. The autopsy revealed that the main cause of death was diffuse alveolar damage (DAD). Interstitial pneumonia related to dermatomyositis is not histologically uniform; the response to the therapy depends on its histological type. The patients with dermatomyositis who have poor prognosis are clinically characterized by acute onset with general symptoms and less pronounced muscle weakness; they generally show DAD in their lungs. We need to establish a simple method for distinguishing histological types of interstitial pneumonia and adequate therapy for each one.