Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Auto-immune mechanisms, mostly cell mediated, have been described as the basic abnormality in polymyositis and dermatomyositis. We report here the case of a patient affected with dermatopolymyositis, resistant to therapy, who was treated with corticosteroids and cyclosporin. She presented with an infiltrative, erythematous rash on the neck, eyelids and cheeks and weakness of the proximal limb and cervical muscles. The dramatic and persistent response to cyclosporin obtained in this case represents a clear demonstration of the action of this drug in cases of dermatomyositis.
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PMID:Clinical effects of cyclosporin in dermatomyositis. 234 3

Skin eruptions with features of subacute cutaneous lupus erythematosus occurred in a patient with dermatomyositis who was taking griseofulvin. The patient had antibodies against SSA/Ro and SSB/La antigens. Skin rashes disappeared after discontinuation of griseofulvin and recurred on challenge with the drug. These findings support previous studies that the photoactive drug may be synergistic with anti-SSA/Ro antibody to produce lesions of subacute cutaneous lupus erythematosus.
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PMID:Subacute cutaneous lupus erythematosus lesions precipitated by griseofulvin. 247 12

We examined retrospectively the use of hydroxychloroquine in 9 patients with childhood dermatomyositis (DM). These patients had an incomplete response to corticosteroid therapy or had disease exacerbation when attempting to taper corticosteroids. They received hydroxychloroquine in a mean dosage of 3.6 mg/kg (range 2-5 mg/kg) while maintaining corticosteroid therapy. Significant improvement was noted at 3 months of therapy in rash (p less than 0.0001), proximal (p = 0.0004) and abdominal muscle strength (p = 0.0001) and at 6 months of therapy in reduced prednisone dosage (p less than 0.02). These data suggest hydroxychloroquine may be helpful in some patients with childhood DM, particularly those with prominent cutaneous disease or significant corticosteroid toxicity.
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PMID:Adjunctive use of hydroxychloroquine in childhood dermatomyositis. 772 68

A 49-year-old woman was admitted in February 1987, with a six-month history of joint pain, maculopapular and erythematous rash, proximal muscle weakness and a two-month history of skin ulceration and dyspnea on exertion. Physical examination showed Gottron's papules on her fingers and a faint heliotrope rash. Biopsy of erythematous skin lesions on the shoulder and the back of the hand revealed perivascular inflammatory cell infiltration and tiny ulcerative lesions of the cutaneous tissue. Biopsy of the right quadriceps muscle showed type II fiber atrophy and slight perivascular lymphocytic infiltrate, whereas serum CPK level was within normal range. Chest X-ray film showed granular infiltrates in both lower lung fields. Based on the current findings the case was diagnosed as dermatomyositis associated with interstitial pneumonia. The administration of prednisolone, 30 mg/day resulted in the improvement of the skin lesions and muscle weakness, while the intensity of lung infiltrates was little affected. Three months after steroid therapy, the patient was readmitted because of increasing dyspnea on exertion and multiple skin ulcers. Chest X-ray revealed a small amount of gas in the mediastinum, in addition to slight deterioration of interstitial lung disease. In spite of various treatments for pneumomediastinum, including bed rest, administration of analgesics and oxygen inhalation, it developed rapidly, complicated severe subcutaneous emphysema and right-sided pneumothorax. Although high-dose prednisolone therapy and mediastinal drainage were performed, the pneumomediastinum was not resolved and she died from respiratory failure. At autopsy, predominant histological features of the lungs were acute interstitial pneumonia with hyaline membrane and edematous granulation formation in the alveoli.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of dermatomyositis which rapidly developed to respiratory failure in the presence of pneumomediastinum]. 268 9

Dermatomyositis in childhood is an uncommon disease, affecting muscle and skin. The disease usually has an insidious onset; the proximal muscle groups are classically more affected than the distal group. If left untreated, the disease will either spontaneously arrest or will progress until the child is completely bedridden, with death secondary to hypoventilation and aspiration. For a definitive diagnosis 3 or 4 of the following criteria (plus rash) are required: 1) symmetrical limb girdle weakness; 2) muscle biopsy evidence of myositis and muscle necrosis; 3) elevation of muscle enzymes; 4) electromyographic changes of myositis. The main pathologic feature of juvenile dermatomyositis is vasculitis affecting small arteries and veins of muscle, skin and gastrointestinal tract. Whether muscle from patients with polymyositis contains a specific auto antigen or is contaminated with an immunogenic infectious agent such as a virus (coxsackie virus, for instance) remains unclear. Childhood dermatomyositis is almost uniformly responsive to steroid treatment; there is a good chance of remission with minimal risk of secondary complications with an initial low dosage of prednisone (1 mg/kg/day). The use of additional drugs such as azathioprine, methotrexate or cyclophosphamide is reserved for patients who are either not completely responsive to steroids or difficult to wean off steroids. Cyclosporine A has been proposed to achieve a reduction in steroid dosage.
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PMID:[Dermatomyositis in children]. 271 39

A muscle biopsy established the diagnosis of dermatomyositis in a 22-year-old woman with normal muscle enzyme levels, 18 months after she presented with constitutional symptoms, a rash and polyarthritis. Her subsequent course was complicated by cutaneous ulcers with histological changes suggesting vasculitis, interstitial pulmonary involvement and recurrent pneumomediastinum, the latter being recently reported in 2 other cases of dermatomyositis. Cutaneous vasculitis has been present in all cases and it is suggested that the pneumomediastinum may be due to pulmonary vasculitis.
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PMID:Recurrent pneumomediastinum in adult dermatomyositis. 272 55

We examined the relationships between the clinical features and outcome of 43 patients with polymyositis-dermatomyositis (PM-DM) and muscle biopsy findings, with specific reference to the pattern of distribution of inflammatory cells. Perifascicular inflammation was associated with the presence of the rash of DM. No relationships were found between the other patterns of distribution of inflammatory cells in muscle and various clinical findings in patients with PM-DM, or the clinical course of the disease. Vasculitis of large vessels (greater than or equal to 20 micron) was seen in only 5 cases (11%) and was not present in childhood PM-DM.
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PMID:Relationships between clinical features and distribution of mononuclear cells in muscle of patients with polymyositis. 319

A 31-year-old woman with long-standing amyopathic dermatomyositis developed a maculopapular rash resembling erythema multiforme in some areas, accompanied by lymphadenopathy and malaise. An acute infection with Toxoplasma gondii was found to be the cause of clinical symptoms that mimicked an exacerbation of the underlying condition.
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PMID:[Toxoplasma infection in dermatomyositis]. 323 39

Sixteen cases of dermatomyositis-polymyositis in children (dermatomyositis 15; polymyositis 1) have been reviewed. Proximal muscle weakness with a characteristic skin rash was vital for diagnosis in the majority of children. The positive laboratory tests such as creatine phosphokinase (CPK), electromyography (EMG) and muscle biopsy were helpful but results were normal in some cases even in the acute phase of the illness. All but one had been treated initially with high dose corticosteroids followed by a low dose maintenance for a prolonged period. Two in addition had had cytotoxic agents. Thirteen of the sixteen were currently in remission. In the other three cases, one with polymyositis only, the disease remained active and the two with dermatomyositis died. Only three of the patients were incapacitated by residual fixed contractures and extruding calcinosis. On the whole the prognosis of dermatomyositis is good.
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PMID:Dermatomyositis-polymyositis in children. 351 51

An effort was made to identify all patients with polymyositis/dermatomyositis (PM/DM) admitted to hospitals in Israel from 1956-1976. The diagnosis of PM/DM was retrospectively reviewed in 92 (46 definite, 26 probable, and 20 possible) cases. The most common complaints and physical findings in the course of the disease were muscle weakness (86 patients), rash (53 patients), arthritis or arthralgia (39 patients), and dysphagia (35 patients). Elevated serum aldolase levels were found in 64% of the patients for whom data were available; 92% had abnormal electromyogram results, and 60.9% had muscle histopathology consistent with PM/DM. Malignancy was diagnosed in 13 patients. Malignancy, ischemic heart disease, and pulmonary complications were the most common causes of death. The actuarial survival curve was heterogeneous, with an accelerated mortality during the first year after diagnosis and a slower mortality during the following 7 years. Independent unfavorable prognostic signs were: failure to induce remission, leukocytosis, fever, older age, a shorter disease history, and dysphagia.
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PMID:Prognostic factors in polymyositis/dermatomyositis. A computer-assisted analysis of ninety-two cases. 397 73


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