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 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over 100 cases of disorders closely resembling classic autoimmune diseases have been reported among patients who were injected or implanted with a diverse group of chemicals including paraffins, vegetable oils or silicone. Most cases have occurred in silicone breast implant recipients, especially those who received their prostheses 2-10 years prior to onset of symptoms. A high proportion of patients exhibit classic signs and symptoms of Sjogren's syndrome or scleroderma. Affected patients typically experience some combination of fatigue, myalgia, joint pain, sicca syndrome (dry eyes and mouth), synovitis, rash, alopecia, muscular weakness or lymphadenopathy, and autoantibody formation. Less commonly, patients may have the CREST syndrome (calcinosis, Raynaud's phenomena, esophageal hypomotility, sclerodactyly and telangiectasias), hypertension, pulmonary fibrosis, or central nervous system pathology.
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PMID:Silicone-reactive disorder: a new autoimmune disease caused by immunostimulation and superantigens. 828 1

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.
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PMID:Dermatomyositis exacerbated by abdominal Marlex mesh implantation: adjuvant effect? 892 88

Calcinosis cutis in dermatomyositis is dystrophic calcification appearing late in the course of the disease. Two cases are reported here of calcinosis cutis that presented years before other clinical manifestations of juvenile dermatomyositis. The first case was a 14-year-old Thai girl who had asymptomatic subcutaneous nodules that spontaneously ruptured, exuding a chalky discharge and healing with an atrophic scar 8 years before the onset of other clinical manifestations of juvenile dermatomyositis; that is, Gottron's papules, proximal muscle weakness grade IV/V with atrophy, slightly elevated serum creatinine phosphokinase level and an abnormal electromyogram compatible with myopathy. The second case was a 15-year-old Thai boy who had calcinosis cutis 3 years before the onset of other clinical manifestations of juvenile dermatomyositis, and the calcinosis cutis was so severe that it interfered with the movement of his extremities. Both cases responded well to aluminium hydroxide therapy.
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PMID:Calcinosis cutis presenting years before other clinical manifestations of juvenile dermatomyositis: report of two cases. 943 16

A retrospective analysis of 25 Arab patients with juvenile dermatomyositis (JDMS) was conducted between 1988 and 1996. The mean age at disease onset was 8.25 years (range 1.5-15 yrs) with a male: female ratio of 1.5:1. The disease duration before diagnosis was 1-108 months. Two patients had a family history of JDMS. The clinical features included fever in 14 patients (56%), weight loss in 20 (80%), muscle weakness in all 25 (100%), and muscle pain in 14 (56%). Skin lesions included Gottron's papules in 15 patients (60%), heliotrope in 13 (52%), erythematous malar rash in 8 (32%), and pigmentary changes in 12 (48%). Seventeen of the 25 patients had arthralgia (68%) and 16 patients had arthritis (64%). Gastrointestinal symptoms were noted in 19 patients (76%). Myocarditis with cardiac failure was the initial presentation of 1 patient, while 2 had conduction defect. Twelve patients (48%) had respiratory symptoms. The course of the disease was complicated by calcinosis in 10 patients (40%). All of the patients were treated with prednisone; 15 were also treated with methotrexate. The duration of follow up ranged from 6-108 months (mean 54.5 months). Twenty-three patients improved, including those who had calcinosis at the time of presentation, with a current muscle power of 4/5 in 10 patients (40%) and 5/5 in 13 patients (52%). No deaths were reported in our series and no patients are currently bedridden.
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PMID:Juvenile dermatomyositis: clinical profile and disease course in 25 patients. 1008 45

A 22-year-old male with juvenile dermatomyositis presented with fever up to 40 degrees C and acute pain in his right thigh accompanied by muscle weakness, a skin rash and a tender swelling. Serum aspartate aminotransferase (AST) and aldolase were mildly elevated. C-reactive protein (CRP) and fibrinogen were markedly increased. The differential white blood cell count revealed relative lymphopenia. Radiography showed diffuse calcifications particularly around the thighs and knees of both legs. Magnetic resonance imaging (MRI) demonstrated inflammatory infiltrates in the right thigh. The lesions were identified as phlegmone by immunoszintigraphy with 99mTc-labelled antigranulocyte antibodies. On the 10th day of treatment Staphylococcus aureus was cultured from blood. Patients with juvenile dermatomyositis and calcinosis may develop bacterial infections of soft tissue which sometimes mimic a disease flare. For differential diagnosis plain radiographs, CT scans and MRI are of limited value. Immunoszintigraphy is able to differentiate between infiltrates caused by granulocytes and lymphocytes.
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PMID:[Juvenile dermatomyositis--acute recidivism or sepsis?]. 1041

Juvenile dermatomyositis is a rare, chronic multisystemic inflammatory disorder of unknown etiology, characterized by a typical skin rash and proximal muscle weakness. A retrospective study from the medical records of patients diagnosed as juvenile dermatomyositis was performed at Queen Sirikit National Institute of Child Health from 1988 to 1998. There were seven cases of juvenile dermatomyositis diagnosed according to the criteria of Bohan and Peter. Six cases were female and one case was male. The age of diagnosis ranged from 2.5 years to 11 years. (mean age was 7 +/- 3.6 years). The presenting symptoms were muscle weakness (6 cases), muscle pain (2 cases) and skin rashes (4 cases). All of the patients developed proximal muscle weakness of the lower extremities varying from grade 3 to grade 4. The cutaneous manifestations were heliotrope signs (6 cases), gottron's papules (2 cases), photosensitivity (2 cases) and calcinosis cutis (4 cases). Electromyography (EMG) was performed in 6 cases and revealed typical change of myopathic type. Elevated muscle enzymes were noted in all cases. Muscle biopsy was performed in 6 cases and was compatible with myositis. Oral prednisolone (1-2 mg/kg/day) was given in 6 cases and the muscle weakness improved. There was no mortality in this study. Four cases developed calcinosis cutis 1 to 3 years after muscle weakness and did not respond to any treatment. In conclusion, juvenile dermatomyositis is a disease which causes chronic disability in children. Early diagnosis and treatment can prevent morbidity and mortality. Calcification at the skin usually occurs after the onset of muscle weakness several months to years after diagnosis.
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PMID:Juvenile dermatomyositis in Thai children. 1185 94

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.
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PMID:[Dermatomyositis and polymyositis: clinical aspects and treatment]. 1196 87

The clinical features, outcome and complications of juvenile dermatomyositis were studied in a tertiary care hospital by retrospective analysis of case records. Nineteen patients were treated over an 11-year period. Median age at diagnosis was 12 years (2.5-16 years). Median duration of disease prior to diagnosis was 12 months (2-96 months). Proximal muscle weakness was seen in all 19 cases, neck muscle weakness in 14, pharyngeal muscle involvement in 5 and respiratory muscle involvement in 3 cases. Heliotrope rash was seen in 9 and Gottrons rash in 8 patients. Myocarditis and GI bleed were seen in 1 each while interstitial lung disease was seen in 2 patients. All except one patient received prednisolone. Methotrexate was used in 13 and azathioprine in 3 patients. Eight patients are in complete remission (CR), 8 partial remission and 2 patients had no response. Complications were calcinosis in 5, contractures in 2, TB in 4 and pyogenic infections in 4 patients. Juvenile dermatomyositis needs to be recognised early and treated aggressively to improve outcome.
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PMID:Outcome in juvenile dermatomyositis. 1242 38

Although soft tissue calcifications are well known to occur as a late manifestation in scleroderma, symptomatic paraspinal calcinosis is very rare. Clinically, patients present with focal neck pain, weakness or radiculopathy, and decreased range of motion of the neck. We describe the imaging features of a rare case of cervical paraspinal calcinosis in a 74-year-old woman with long-standing scleroderma. Standard radiography is usually sufficient to confirm the diagnosis, but CT-scan allows a more precise location of the calcifications around the facet joints, sometimes with associated erosions. The advantage of MRI is to evaluate the possible intraspinal extension of the calcifications in case of focal neurological symptomatology.
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PMID:Paraspinal cervical calcifications associated with scleroderma. 1283 21

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.
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PMID:[Juvenile dermatomyositis]. 1705 62


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