hypomyopathic dermatomyositis

  • 文章类型: Case Reports
    重叠综合征是一项临床挑战,为治疗医师带来了广泛的治疗选择。解决患者的每一个投诉至关重要。一名50岁的女性患者出现皮肤增厚,变黑,角化过度;吞咽困难;关节痛;肌病的特征;雷诺现象;和口干。炎症标志物随着高尔基体模式的抗核抗体(ANA)阳性而升高,抗干燥综合征相关抗原A(抗SSA)/Ro603+,抗SSA/Ro523+,和提示重叠综合征的抗PM/Scl2+抗体。尽管患者没有呼吸道疾病,在评估过程中发现了一种独特的间质性肺病(ILD)模式.皮肤表现令人费解,但对来自两个不同部位的皮肤活检的组织病理学分析显示了皮肤狼疮和皮肌炎的显著特征。用羟氯喹治疗,毛果芸香碱,硝苯地平,甲氨蝶呤,和外用他克莫司在临床特征上产生了显著的改善。此病例突出了不同自身免疫性疾病的微妙和花哨的特征。角化过度的皮肤变化是最显著的特征,但整个评估过程揭示了已知自身免疫性疾病的许多罕见表现,这些表现可以为我们对结缔组织疾病(CTDs)的新认识领域打开大门.我们的病例报告显示了ANA模式的显著异质性,ILD模式,临床表现,和治疗方法。
    Overlap syndrome is a clinical challenge and brings together a wide range of treatment options for the treating physician. Addressing each and every complaint of the patient is crucial. A 50-year-old female patient presented with skin thickening, blackening, and hyperkeratosis; dysphagia; joint pain; features of myopathy; Raynaud\'s phenomenon; and dry mouth. Inflammatory markers were raised along with a positive antinuclear antibody (ANA) with Golgi apparatus pattern, anti-Sjögren\'s-syndrome-related antigen A (anti-SSA)/Ro60 3+, anti-SSA/Ro52 3+, and anti-PM/Scl 2+ antibodies that suggested overlap syndrome. Although the patient had no respiratory complaints, a unique interstitial lung disease (ILD) pattern was noted during the evaluation. Skin manifestations were puzzling, but the histopathology analyses of skin biopsies taken from two different sites revealed distinguishing features of cutaneous lupus and dermatomyositis. Treatment with hydroxychloroquine, pilocarpine, nifedipine, methotrexate, and topical tacrolimus produced a dramatic improvement in the clinical features. This case highlights subtle and florid features of different autoimmune diseases. The hyperkeratotic skin changes were the most striking feature, but the whole evaluation process unveiled many rare presentations of known autoimmune conditions that can open doors to new areas of our understanding toward connective tissue diseases (CTDs). Our case report demonstrates significant heterogeneity in the ANA patterns, ILD patterns, clinical manifestations, and treatment approaches.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    BACKGROUND: Clinically amyopathic dermatomyositis (CADM) is characterized by typical skin lesions with no (amyopathic) or subclinical (hypomyopathic) evidence of muscle involvement. Patients with CADM may also develop rapidly progressive interstitial lung disease (ILD), and have a poor prognosis. However, the diagnosis of rapidly progressive ILD faces a challenge during the severe acute respiratory syndrome coronavirus 2 pandemic. Severe acute respiratory syndrome and ground-glass attenuation on a chest computed tomography scan are the presenting features in both conditions.
    METHODS: A 45-year-old woman with amyopathic dermatomyositis had acute onset of fever and dyspnea in February 2020. She had abnormal lung findings on CT scan. Polymerase chain reaction testing for SARS-CoV-2 was not available at that time. Chest CT revealed non-specific manifestations that could be either the signs of ILD or SARS-CoV-2 infection. Antiviral therapy was initiated with oseltamivir. Three days later, she had erythema on face, palm, and back. The ratio of lactate dehydrogenase (LDH) isoenzyme 3 to total LDH was elevated. The ratio of LDH isoenzyme 1 to total LDH was declined. Therefore, she was transferred to the rheumatology ward for further treatment. However, she died from respiratory failure 2 weeks later.
    CONCLUSIONS: We speculate that the altered LDH isoenzyme pattern may be an early biomarker for co-occurrence of CADM and ILD.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    Dermatomyositis is associated with malignancies and is known to have systemic involvement. However, associations with bone diseases have not been well described in the current literature. This article describes the second reported case of the co-existence of dermatomyositis and Paget\'s Disease of Bone (PDB), but is the first report to describe such co-existence in a specific subtype of dermatomyositis - hypomyopathic dermatomyositis. Our patient was a 51-year-old woman who presented with prolonged fever, myalgia, morning stiffness, and rashes pathognomonic of dermatomyositis. There was no muscle weakness clinically, although muscle enzymes were raised and electromyogram revealed myopathic changes. Further imaging showed the incidental finding of a T11 vertebral bone lesion, of which biopsy confirmed the diagnosis of PDB. Our report illustrates the diagnostic approach to bone lesions in patients with dermatomyositis, and takes a closer look at the pathophysiology and management implications of the co-occurrence of these two rare diseases.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    低肌病性皮肌炎(DM)表现为与皮肌炎一致的皮肤病变,但没有临床上明显的肌肉无力。与伴随的肌肉受累相比,皮肤表现通常是难治性的,并且对常规治疗更具抵抗力。我们介绍了一名61岁的DM低肌病性患者,该患者对标准疗法无反应,但成功接受了低剂量白介素2(IL-2)的治疗,没有明显的副作用。我们得出的结论是,低剂量IL-2是低肌病性DM的安全有效治疗方法。
    Hypomyopathic dermatomyositis (DM) presents with cutaneous lesions consistent with dermatomyositis but in the absence of clinically appreciable muscle weakness. The cutaneous manifestations are often refractory and more resistant to conventional therapy than concomitant muscle involvement. We present a 61-year-old hypomyopathic patient with DM who failed to respond to standard therapy but was successfully treated by low-dose interleukin-2 (IL-2) with no significant side effects. We conclude that low-dose IL-2 is a safe and effective treatment for hypomyopathic DM.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    A 47-year-old Haitian male with no known past medical history was admitted to the hospital for gradually progressive dyspnea, nonproductive cough, and weight loss. He also endorsed a one-year history of joint pains. He was febrile and tachycardic and in mild respiratory distress. Other pertinent physical examination findings included diffuse inspiratory crackles, digital ulcers, and symmetric swelling of the wrists, elbows, shoulders, and knees. He was found to have a right basilar consolidation on chest computed tomography (CT) and was placed on antibiotics for presumptive pneumonia. His CD4 count was 158 cells per microliter despite testing negative for human immunodeficiency virus (HIV). A thorough infectious workup was unrevealing, and he did not improve with antibiotics. He had a weakly positive anti-nuclear antibody (ANA) with an otherwise negative rheumatologic workup. Creatinine kinase and aspartate aminotransferase were mildly elevated in the absence of overt muscle weakness. A myositis panel, including melanoma differentiation-associated protein five (anti-MDA5) antibody, was negative at the time. He was discharged on a short course of prednisone without a definitive diagnosis. He returned several months later with worsening respiratory symptoms. At this time, a lung biopsy revealed interstitial lung disease. Repeat myositis panel demonstrated anti-MDA5 positivity. The patient was also found to have new-onset non-ischemic heart failure with reduced ejection fraction. A diagnosis of hypomyopathic dermatomyositis was made based on clinical, laboratory, and imaging findings. The patient was restarted on prednisone, and mycophenolate mofetil was subsequently initiated for maintenance therapy.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号