SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A case of juvenile dermatomyositis developing intermuscular fascial plane calcification in the thighs early in the course of the disease is described. The use of CT was helpful in localizing the calcium deposits between the muscular layers before surgical removal of the calcification. These procedures may have prevented fistulization and development of contracture of the joints.
We describe a 35-year-old woman with dermatomyositis, who four months after implantation of abdominal Marlex mesh, developed a severe exacerbation of her disease with muscle weakness, elevated acute-phase reactants, a high level of muscle enzymes, and the appearance of dermal lesions with calcinosis. The Marlex mesh implant may have triggered the flare-up of her underlying autoimmune disorder.
A two years, ten months old male with dermatomyositis was anaesthetized with enflurane, nitrous oxide and oxygen by mask followed by intravenous succinylcholine to facilitate endotracheal intubation. The evoked thumb twitch in response to succinylcholine demonstrated an abnormal, short-lived contracture. The depression, duration and return to control of muscle twitch tension and a transient rise in serum potassium concentration followed a normal pattern.
Dermatomyositis (DM) is a rare disease of unknown aetiology involving weakness of proximal muscles, myalgia and impairment of the integument. Due to its low incidence, only few reports exist on the anaesthesiologic management of neuromuscular blockade in patients with DM considered for surgical therapy. In this setting, special attention should be given to the administration of muscle relaxants since an increased sensitivity to these drugs in neuromuscular diseases may be expected. METHODS. This case report describes the neuromuscular monitoring of a female patient aged 53 years with DM undergoing strumectomy and treated with methotrexate and prednylidene. Muscle relaxation was induced with 0.35 mg/kg atracurium (1.5 x ED95). An additional bolus of 0.07 mg/kg was administered 50 min later. Control of sufficient relaxation was performed by tactile methods, and time of onset (TO), duration of action (DA) and recovery index (RI) were measured. RESULTS. Relaxation was obtained within TO of 3 min, DA of 50 min and RI of 11 min. These timer indicate slightly increased sensitivity to atracurium. The postoperative outcome demonstrated no muscular weakness and sufficient spontaneous ventilation. DISCUSSION. To date, few experiences of neuromuscular blockade with atracurium in DM have been presented, hence this report. In addition, no standard recommendations are given regarding the application of nondepolarizing muscle relaxants in this context. In our case, atracurium could be implemented as a safe drug under neuromuscular monitoring.
We report on a 70-year-old female patient with dermatomyositis. The connective tissue vascular bed was studied with capillaroscopy to evaluate morphological and functional changes of skin capillaries. Capillaroscopic findings showed morphological alterations such as rarefication of capillaries, avascular fields and tortuosity of nailfold capillaries. In addition, fluorescence-videomicroscopy showed pathologically increased transcapillary diffusion of dye in the apex region of nutritive skin capillaries. Increased capillary leakage probably contributed to the development of oedema in our patient. Immunosuppressive therapy combined with intensified manual lymphatic drainage was successful.
Dermatomyositis, polymyositis, inclusion body myositis and myositis overlap syndromes are systemic immune disorders of unknown origin with muscle weakness and elevated values of creatinkinase in the serum. Muscle biopsy is pivotal for a proper clinical diagnosis. Extramuscular findings at the skin, the joints or internal organs (lung, heart) are characteristic for the different clinical presentations of dermato- or polymyositis and are usually absent in inclusion body myositis. With the exception of inclusion body myositis myositis-associated autoantibodies are frequently present and associated with distinct clinical manifestations (e. g. antisynthetase syndrome). The rate of malignancy is elevated for several years after onset of myositis. Especially in polymyositis an appropriate differential diagnosis of infectious, endocrine, metabolic or neuromuscular causes of muscle disease is necessary. Glucocorticosteroids are the first choice of treatment in dermato- or polymyositis. Methotraxate, azathioprine, cyclophosphamamide, i.v. immunoglobulins and other drugs are used in diseases courses with continuous high dose requirement of corticosteroids.
Children with dermatomyositis may have extensive subcutaneous and intermuscular calcium-laden fluid collections referred to as "milk of calcium." The distinctive MR appearance of such collections in an upper extremity of a 16-year-old girl is presented. MR can differentiate these collections from abscesses and guide appropriate therapy.
The group of autoimmune cutaneous connective tissue diseases (CCTD) includes systemic sclerosis, dermatomyositis (DM), lupus erythematosus (LE), and subtypes. Most CCTD patients can be assigned to one of these phenotypes or mixed forms, and transitions among the subsets exist. We present the case of a 26-year-old woman with bullous LE and a background of pre-existing DM. Observations of transitions between DM and LE are extremely rare. Nevertheless, this case demonstrates the close relationship between these two disorders in the context of autoimmune connective tissue disease.
The aim of this study was to evaluate outcome in children with juvenile dermatomyositis (JDMS) at a tertiary care center in North India and have a long term follow-up. Medical records of children with JDMS managed at a tertiary care hospital were reviewed during a 13-year period to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modalities used and (3) long term functional outcome. Thirty three patients diagnosed with JDMS met the inclusion criteria. Mean age at diagnosis was 8.7 +/- 3.3 years. Mean duration of disease prior to treatment was 1.18 years (range 1 month-5 years). The total follow-up period was 136.7 patient-years. Immunosuppressive therapy was given in 31/33 and a distinct monocyclic course was seen in 72.7% cases. Lipodystrophy was seen in 10/33 (30.3%), calcinosis in 7/33 (27.3%), cutaneous ulcers in 6/33 (18.2%), dysphagia in 5/33 (15.2%), and contractures in 4/33 (12.1%) cases. A steady and sustained response was seen in patients who had received "adequate" doses of steroids at the time of initiation of treatment. Methotrexate, hydroxychloroquine, azathioprine and intravenous immunoglobulin were used in patients with poor response to corticotherapy. There were two deaths in our series. Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long-range sequelae of JDMS. Our patients seem to have a different clinical profile on follow-up as compared to series published from the West.
Here we report two patients with severe juvenile dermatomyositis (JDM) complicated with extra musculocutaneous involvement. The first case (a 10-year-old boy) had unusual initial presentation of JDM complicated with interstitial lung disease documented with high-resolution computed tomography. He had a rapidly progressive course and died in 7 weeks after the onset of the disease despite steroid and immunosuppressive treatment. The second case (a 14-year-old boy) was presented with myositis complicated with hepatitis. He also had a chronic course of JDM with unfavorable outcome. It appears that the prognosis of patients with severe JDM is related with the degree of autoimmune vasculitis on extra musculocutaneous involvement.
We report a case of a 70-year-old man with dermatomyositis complicated by esophageal involvement resistant to corticosteroid therapy. It had serious and potentially life-threatening implications, resulting in impossible oral feeding. This condition was successfully resolved by administration of cyclosporine A.
A 42-year-old man with dermatomyositis presented with right-upper-quadrant dull pain and normal blood pressure for 10 days. Abdominal ultrasonography, abdominal computed tomography, and angiography revealed a retroperitoneal tumor with direct invasion to the liver and the diaphragm. The diagnosis of nonfunctioning malignant pheochromocytoma was made on the basis of clinical evidence. The tumor was removed en bloc with part of the diaphragm, the right lobe of the liver, the right adrenal gland, and the right kidney. Pathologic examination with immunohistochemical staining revealed a malignant pheochromocytoma growing exophytically from the right adrenal gland and invading the right lobe of the liver and the diaphragm. The postoperative course has been uneventful and no recurrence has been noted over a 6-month follow-up period.
Juvenile dermatomyositis (JDM) is an inflammatory multi-system disease of unknown etiology with classic involvement of the skin and striated muscles. Following a prodromal period, patients develop a progressive proximal muscle weakness. Typical skin involvement includes heliotrope rash, facial erythema, Gottron's sign and nailfold capillary abnormalities. For the diagnosis of JDM, modified Bohan and Peter criteria are used including clinical skin and muscle signs plus elevated muscle enzymes and typical findings from electromyography, muscle biopsy and - more recently - also on magnetic resonance imaging. Steroids are administered classically as high-dose oral treatment. Intravenous pulse therapy with intermittent lower dose oral treatment and other immunosuppressive drugs such as methotrexate may reduce steroid side-effects. Prognosis in JDM has improved, and most patients eventually make a full functional recovery. However, a few patients still die from their disease, and in a minority significant sequelae with muscle atrophy or severe calcinosis ensue.
We report a 57-year-old male with myasthenia gravis complicated with dermatomyositis and rheumatoid arthritis without evidence of thymoma. He showed prominent muscle wasting and weakness in the four extremities and trunk in addition to swallowing disturbance. He showed intolerance to exercise on a bicycle ergometer, and muscle biopsy specimens demonstrated ragged-red fibers. An anti-acetylcholine receptor (AChR) antibody was detected in his serum but no anti-mitochondrial M2 component antibody was found. In contrast, results of immunohistochemical study indicated that his serum sample reacted to muscle mitochondria as well as AChR. These results indicate the presence of an unidentified anti-mitochondrial antibody that may be involved in the development of mitochondrial dysfunction in skeletal muscle of the present patient.
We evaluated the expression of tumor necrosis factor-alpha (TNF-alpha) mRNA in muscle biopsy specimens from patients with polymyositis (PM) and dermatomyositis (DM) to clarify its role in the pathogenesis of PM and DM. We performed non-radioactive in situ hybridization studies for TNF-alpha combined with immunohistochemistry for cell type-specific markers on muscles from ten PM and five DM patients. TNF-alpha-positive infiltrating cells present in the endomysium and perimysium were found in all PM and DM muscles. The frequency of TNF-alpha-positive cells against total infiltrating cells was similar among PM and DM (27.1 +/- 7.4% in PM and 28.5 +/- 13.6% in DM). However, TNF-alpha/CD8-positive lymphocytes and TNF-alpha-positive macrophages invading the non-necrotic muscle fiber were observed only in PM but not in DM. TNF-alpha was more highly expressed in PM and DM than was previously thought, and it was suggested that TNF-alpha plays a role in muscle fiber degeneration in PM.
We report a rare association of juvenile dermatomyositis with subclinical hypothyroidism due to auto-immune thyroiditis. Although the patient had no symptoms of hypothyroidism, thyroid auto-antibodies remained elevated even after disease remission.
Dermatomyositis (DM) is a rare inflammatory disorder of the skin and muscles associated with an increased incidence of malignancy. We describe herein the case of a 59-year-old woman with DM accompanied by rectal cancer. Following excision of the rectal cancer, the characteristic features of the skin rash such as the heliotrope eyelid rash and Gottron's papules, and proximal muscle weakness, improved. Moreover, the elevated preoperative serum levels of muscle-associated enzymes, including aspartate transaminase, creatine phosphokinase, lactate dehydrogenase, and aldolase, decreased from 38 to 16 (IU/1), 138 to 42 (IU/1), 672 to 515 (IU/1), and 32.2 to 4.3 (IU/1), respectively. The current concepts of the correlation between DM and malignancy are discussed with regard to the present case.
We describe a rare case of a 46-year-old Japanese man with dermatomyositis (DM) and interstitial lung disease who developed spontaneous pneumomediastinum and subcutaneous emphysema. Relatively mild myositis, mild elevation of CK values and the absence of anti-Jo-1 antibody were observed and the case was similar to amyopathic DM. Treatment of this patient with oral prednisolone and cyclosporin A (CsA) was effective for the myositis and interstitial lung disease. The administration of CsA enabled rapid tapering of the dose of prednisolone without aggravating the disease. Pneumomediastinum and subcutaneous emphysema disappeared 5 months later without recurrence. The serum levels of KL-6 were monitored every 2 weeks to help determine whether this may have contributed to the recurrence of interstitial pneumonitis. This is a rare case of pneumomediastinum in a patient with DM.