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PubMed1989

[Dermatomyositis as a paraneoplastic syndrome. A case report].

A female patient with dermatomyositis with the typical clinical findings of this disease, corresponding histological muscular changes and neurological findings is described. Since in the further course of the disease, recurrent gastric carcinoma was diagnosed, this case again emphasizes the significance of dermatomyositis as a paraneoplastic syndrome, and the need for tumor screening when dermatomyositis is diagnosed in advanced adulthood.

Aged↗
PubMed1977

Dermatomyositis in the pediatric patient.

Forty-one children with dermatomyositis who were treated with corticosteroids and who have been followed over a period of 15 years have been presented. Progressive proximal muscle weakness was seen in all and 60% had muscle pain. The skin rash considered classic for dermatomyositis was seen in 33 children at the time of diagnosis. Elevation of serum muscle enzymes, electromyographic abnormalities, and muscle biopsy evidence of acute myositis were of confirmatory diagnostic value. The course of the disease in this study group has reconfirmed the efficacy of adrenal corticosteroid treatment in conjunction with an individualized physical therapy program and consistent followup. Prognosis for life and minimal functional disability has been good. There have been 3 deaths recorded in this series, only one of which was certain in its relationship to dermatomyositis.

Adolescent↗
PubMedDate not supplied

[Toxoplasmosis and dermatomyositis: a causal or casual relationship?].

A case of a 10 year old girl with typical clinical, cutaneous and muscular picture of dermatomyositis and positive serological investigation for toxoplasmosis with neurological complications (hemiparesis and headache) is reported. In literature several case reports have suggested an association between acquired toxoplasmosis and polymyositis-dermatomyositis. A review of previous examples of this association is presented and the possible relationships between the disease are discussed. Although a certain causal relationship is not always established, we suggest that patients with active polymyositis or dermatomyositis should be studied serologically for toxoplasmosis, especially if steroid or immunosuppressive therapy is contemplated. We underline the importance, in these cases, of appropriate antiprotozoal therapy, that, in our experience and in most data reported in other cases, produces clinical benefit.

Child↗
PubMed1989

[Histopathologic aspects of polymyositis and dermatomyositis. Correlation with the clinical course. Study of 57 cases].

Muscle biopsies from 57 patients with dermatomyositis or polymyositis were histologically evaluated and compared with the disease's clinical course. Perifascicular atrophy, perivascular infiltrates and tubular inclusions in endothelial cells were significantly more frequent in young patients with dermatomyositis. On the other hand, in adult polymyositis, which evolves more slowly, necrosis with slight muscular atrophy and perinecrotic infiltrates was observed. This division into two groups was clear when the clinical evolution and histological patterns were compared. The mean age of each group was different, but there was a large overlap. Two different pathogenetic mechanisms can be envisaged: primary involvement of muscle capillaries with muscle ischemia in young patients with dermatomyositis and primary involvement of muscle fibers in adults afflicted with polymyositis.

Adolescent↗
PubMed1989

[Dermatomyositis in children].

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.

Child↗
PubMedDate not supplied

Gastric carcinoma associated with dermatomyositis. Case report.

A case of gastric carcinoma associated with dermatomyositis is reported. Total gastrectomy was performed and subsequently excision of hepatic metastasis. Although the cause-effect relationship between dermatomyositis and internal malignancy remains controversial, the obvious dependence of the clinical course of dermatomyositis on that of the gastric carcinoma in this case suggests a causal involvement.

Adenocarcinoma↗
PubMed1987

Dermatomyositis.

Dermatomyositis is a condition that combines an inflammatory myopathy with characteristic cutaneous disease. This disorder is closely related to polymyositis, which has all the muscular features of dermatomyositits without the presence of skin disease. Both dermatomyositits and polymyositis may occur in the presence of other collagen vascular diseases such as lupus erythematosus, scleroderma, Sjögren's syndrome, rheumatoid arthritis, and various vasculitides. Dermatomyositis seems to be characterized by an increased frequency of internal malignancy and both dermatomyositis and polymyositis are associated with morbidity and mortality. Therefore, prompt and aggressive therapy is necessary.

Dermatomyositis↗
PubMed1988

[A case of dermatomyositis associated with breast cancer].

Presented is a case of a 48-year-old woman with dermatomyositis associated with breast cancer. A standard radical mastectomy against the breast cancer was performed in addition to the administration of adriamycin, 5-Fu, and tamoxifen as adjuvant chemoendocrine therapy. The symptoms of dermatomyositis were not that severe, so no steroid therapy was given. The patient now is ambulatory and has been discharged, and no symptoms of recurrence or exacerbations of dermatomyositis has occurred.

Adenocarcinoma↗
PubMed1987

[Renal manifestations in dermatomyositis and polymyositis].

Renal involvement is usually considered to be rare in dermatomyositis and polymyositis. The aim of this prospective study was to reevaluate the renal changes which may occur in these two conditions. Twenty patients, 12 with dermatomyositis and 8 with polymyositis of the adult type, were investigated to determine the incidence, the severity and course of renal disease. The 24 hour proteinuria was over 0.1 g in 14 cases and greater than 1 g/24 hours in 6 cases, including one patient with the nephrotic syndrome. Microscopic haematuria was noted in 3 cases and pyuria in 8 cases. Endogenous creatinine clearance was significantly decreased (p less than 0.001) in patients with dermatomyositis compared with a control group of subjects paired for age, sex, weight and serum creatinine concentration. Renal biopsy (5 cases) showed minimal glomerular lesions (2 cases), endocapillary proliferation and extramembranous glomerulonephritis (1 cases) and amyloid deposits (1 case). Although the proteinuria, haematuria and pyuria responded to steroid therapy (except in 1 case), the creatinine clearance remained decreased during the course of steroids.

Adult↗
PubMed1986

[Polymyositis, dermatomyositis and pregnancy: high-risk pregnancy. A further case report and review of the literature].

The authors report a new case where polymyositis/dermatomyositis and pregnancy were associated. The polymyositis and dermatomyositis appeared in the last third of the pregnancy. A caesarean operation had to be performed for fetal distress after 37 weeks of amenorrhoea. An apparently dead small-for-dates baby was born. Progress, however, after resuscitation of the baby was good. At 20 months after delivery the polymyositis and dermatomyositis also were improved. Two and a half years later there was no recurrence. In the light of cases that have previously been reported in the literature, it has been established that on the one hand pregnancy may trigger off the condition and worsen the PM/DM and on the other hand the condition has a bad effect on the progress of the pregnancy. 50% of the babies are born dead or die in the week after delivery and 55% are born prematurely. We discuss the possibility that PM/DM is of viral origin. A confirmation of this is the presence of viral inclusion bodies on electron microscopy found in the muscle cells of the patients who have PM/DM. However, in the previous history of these patients an acute viral myositis in childhood is a troublesome factor. Pregnancy, because it involves alterations in the immune responses of the patients, may lead to a recrudescence of a "sleeping" virus and so be responsible for the appearance or aggravation of polymyositis and dermatomyositis.

Adult↗
PubMed1985

Dermatomyositis and pregnancy.

Dermatomyositis is a rare medical disease complicating pregnancy. Three patients are reported with five successful pregnancies complicated by dermatomyositis. Although pregnancy loss may arise when dermatomyositis complicates pregnancy, successful therapy will permit a satisfactory result. Optimal pregnancy success can be anticipated when pregnancy is undertaken with disease in remission and prospective maternal and fetal evaluation is undertaken.

Adult↗
PubMed1985

Dermatomyositis and Hodgkin's disease. Case report and review of the literature.

The association between dermatomyositis and malignant tumors, especially carcinomas, is well documented. However, lymphoreticular malignancies have rarely been reported. A patient in whom Hodgkin's disease was diagnosed 5.5 years after the onset of dermatomyositis is described. The independent course of the two diseases is discussed and previously reported cases of lymphoma and dermatomyositis are reviewed.

Dermatomyositis↗
PubMed1983

Calcifying dermatomyositis following antitetanus vaccination.

In the course of dermatomyositis, calcinosis is a complication frequently seen in children but rarely seen in adults. Calcinosis often induces poor functional prognosis, reducing mobility of the joints and muscles. Dermatomyositis developed in a 59-year-old woman, following an antitetanus vaccination; the dermatomyositis later became calcifying. To our knowledge, similar cases have not been reported.

Calcinosis↗
PubMed1980

The relationship of dermatomyositis and polymyositis to internal malignancy.

The association of malignancy with dermatomyositis and polymyositis has been questioned. During the last 20 years (1956 to 1975), we have studied 58 cases of myositis that met predefined diagnostic criteria. These cases were analyzed for the frequency of malignancy, prognosis, and the value of a diagnostic test series for malignancy. A significantly greater frequency of malignancy was found with dermatomyositis than with polymyositis. The prognosis of dermatomyositis and polymyositis appears to be altered in the presence of malignancy. In the absence of malignancy, the prognosis is similar in the two forms of myositis. Lastly, the value of a screening laboratory and roentgenographic investigation for the presence of occult malignancy beyond a thorough history, physical examination, and the use of basic laboratory tests such as complete blood count, stool gualac test, urinalysis, multiphasic analysis, and chest roentgenogram was not documented by this study.

Adult↗
PubMedDate not supplied

[Vasculitis in children's dermatomyositis (author's transl)].

Vascular lesions have been discovered by a number of authors in children's dermatomyositis, when they are not ordinary found in adults. Affecting capillaries, arterioles and veinlets, vasculitis appears as an hyperplasia of intima, then as an endothelial necrosis and formation of thrombi. Usually seen with light microscope, these lesions are sometimes only revealed by electron microscope. These vascular lesions are observed in the muscle and mainly in the perimysial interstitial tissue, in the skin, and in the various visceral localisations of the disease: pulmonary, ocular, renal, nervous and mainly digestives. The ulcerations of the digestive tube are due to little infarcts secondary to submucous arteriolar occlusions. The presence of necrotizing microangiopathy in the muscle, in the skin and in the viscera, and the observation by an author of macroglobulin, gammaglobulin and C3 deposits in the vascular walls allows to issue an aetiopathogenic hypothesis. The vascular lesions could be due, at least partly, to immune complex deposits, explained by an allergic mechanism of type III of Gell and Coombs. This process could be released by a viral factor which has to be determined. This pathogeny could bring children's dermatomyositis nearer to the other circulating immune complexe collagenosis: S. L. E., periarteritis nodosa with which many transition form exist, and could separate it to the adults dermatomyositis, which would rather be a disease due to an exacerbation of the delayed hypersensitivity type IV of Gell and Coombs.

Child↗
PubMedDate not supplied

Dermatomyositis and polymyositis in childhood.

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years (range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.

Adolescent↗
PubMed1997

Juvenile amyopathic dermatomyositis.

We report a 15-year-old girl with a 10-year-old history of typical skin features of dermatomyositis (DM) without evidence of muscle involvement. Amyopathic dermatomyositis (ADM) is defined by the presence of biopsy confirmed classic cutaneous findings of dermatomyositis in the absence of any clinical or laboratory signs of muscle disease for at least 2 years after onset of skin pathology. To exclude muscle involvement muscle enzymes should be normal; moreover additional use of magnetic resonance imaging and muscle ultrasound is currently being proposed. It is as yet undetermined, whether early aggressive immunosuppressive treatment of ADM might prevent the development of myositis at a later date or influence the course of the skin disease. In a paediatric patient with ADM we advocate a more expectant attitude with careful and regular monitoring for possible development of muscle disease.

Adolescent↗
PubMed1979

Circulating immune complexes in lupus erythematosus, scleroderma and dermatomyositis.

Circulating immune complexes (CIC) were measured by three different methods in serum from 17 patients with systemic lupus erythematosus (SLE), 3 patients with "hydralazine-induced" SLE-like syndromes, 14 patients with discoid lupus (DLE), 8 patients with systemic sclerosis and 5 patients with dermatomyositis. Immune complexes were detected in 13 of the 17 patients with SLE. All patients with lupus nephritis and typical exanthema had circulating immune complexes. The concentration of immune complexes was inversely correlated to serum complements C4 and C3. All 3 patients with "hydralazine-induced" SLE-like syndromes had circulating immune complexes that disappeared after withdrawal of the drug. Immune complexes were detected in 3 of the 14 patients with DLE; all 3 patients with CIC had wide-spread DLE. In systemic sclerosis, CIC were detected in only 1 of the 8 patients. Four of the 5 patients with dermatomyositis demonstrated CIC in serum. No complement consumption was detected in dermatomyositis and the immune complexes may have been secondary to tissue destruction.

Antigen-Antibody Complex↗