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PubMed1984

[Calcinosis of childhood dermatomyositis. Apropos of 10 cases].

Twenty-eight children were diagnosed as having dermatomyositis (DM) on the basis of the criteria of Bohan and Peter. Ten of the 28 children developed calcinosis. Calcium deposits mainly occurred in DM with gradual onset of symptoms and with a chronic course. Calcinosis always appeared insidiously and early during the active stage of the disease. It persisted for a long time beyond the remission of the active muscle symptoms without tendency to spontaneous resolution. Long-term follow-up data substantiate calcinosis as the most frequent and the most serious sequela in this series of childhood DM.

Calcinosis↗
PubMed1984

Management of polymyositis and dermatomyositis.

The overall prognosis for polymyositis and dermatomyositis has improved with the judicious use of corticosteroids, while azathioprine and other cytotoxic agents are of value in improving functional ability and decreasing the total steroid requirement. However, the duration of therapy and the relative risks of it must be under constant surveillance. Problems such as respiratory or cardiac failure in the course of the disease are of serious importance and require particular care in management . Finally, the possibility of neoplasm underlying the disease or occurring as a sequel to cytotoxic therapy should always be borne in mind.

Adolescent↗
PubMed1982

Dermatomyositis in childhood.

Childhood dermatomyositis may be a serious and even lethal disease of childhood. Two types are recognized: the rare Banker type, based on vasculopathy and infarction commonly proceeding to death, and the more common Brunsting type, which is a steroid-responsive inflammatory myopathy comparable to the usual adult form. The diagnosis is suggested by the finding of skin changes (present in most patients) and by the results of clinical, enzymatic and electromyographic evaluation of muscle function. A muscle biopsy specimen should be taken in all cases to observe muscle and vessel changes and to rule out unusual myopathy. Direct immunofluorescence of skin and muscle may be helpful. Steroid therapy has reduced inflammation, morbidity and mortality, but remissions may occur without treatment. Methotrexate may be used when there is no response to steroids. Nutrition and physical therapy must be applied properly to be effective. Corticosteroid therapy of the disease has improved the prognosis for musculoskeletal function and for life.

Adolescent↗
PubMed1982

[Bullous dermatomyositis].

The authors present a case of bullous dermatitis with clinical and biological polymyositis. The cause of the blisters can be found in an intense oedema of the superficial and middle dermis and is favoured by liquefaction of the basal layer and fibrinoid degeneration of the superficial dermis. It is diagnosed as dermatomyositis with malignancy even though it has been impossible to confirm the nature of the tumor. The diagnosis is being argued with other diseases that occasionally are vesiculobullous.

Aged↗
PubMed1983

[Periungual capillaroscopy in children with scleroderma and dermatomyositis].

The capillary microscopy is of high value for the early diagnosis of scleroderma: the pattern of megacapillaries is classical in adults. The authors describe the original observation of ramified capillaries in children's with systemic scleroderma (two cases) and dermatomyositis (one case). Such observations lead them to discuss several hypothesis regarding pathogeny (neo-angiogenesis), prognosis and diagnosis of children connectivities.

Adolescent↗
PubMedDate not supplied

Interstitial lung disease in polymyositis and dermatomyositis.

An increased frequency of diffuse interstitial lung disease (DILD) was found in 7 of 15 cases of polymyositis and dermatomyositis seen in a 7-yr period. Affected patients had higher values of creatine phosphokinase and aldolase and more severe muscle necrosis than 8 patients with DILD. Early corticosteroid therapy sufficient to normalize enzymes apparently led to better resolutions of DILD. Active muscle necrosis seemed to be related to the development of DILD.

Adolescent↗
PubMed1982

Juvenile dermatomyositis: medical, social and economic status in adulthood.

Eighteen adults were studied an average of 18.5 years after the diagnosis of juvenile dermatomyositis had been made. Persistent weakness was found in six patients and recurrent rash in the classic distribution in seven. Other clinical features present were Raynaud's phenomenon, arthritis and subcutaneous nodules. Minor increases in the serum creatine phosphokinase level were noted in seven patients and did not correlate with the presence of weakness or rash. Educational achievement and employment status were better in this group than in a group of adults with juvenile rheumatoid arthritis or the general adult population of British Columbia. Significant residual disability related to calcinosis and flexion contractures was present in only three of the patients.

Adolescent↗
PubMedDate not supplied

Polymyositis and dermatomyositis in children: an epidemiologic and clinical comparative analysis.

In an attempt to delineate and compare the clinical, laboratory and epidemiologic features of polymyositis (PM) and dermatomyositis (DM) in children, the records of all 19 hospitals in Memphis and Shelby County, Tennessee, were surveyed over the 25-year period, 1948-1972. Seventeen documented cases of PM and 26 of DM were identified over the total period. PM was not diagnosed prior to 1962, while DM was found throughout the study. A significant female excess was observed in the PM group and in the combined patients 10-19 years of age. The only major differences found between PM and DM were the more acute and severe muscle involvement in DM and its cutaneous manifestations. Juvenile PM is a definite entity which should be differentiated from other myopathies because of its favourable response to corticosteroid therapy.

Adolescent↗
PubMed1981

[Digestive manifestations of dermatomyositis (author's transl)].

The principal digestive anomalies detected by radiological examination in dermatomyositis are the motor and functional consequences of the smooth and striated muscle lesions in these tissues. Vascular changes cause ulcerations, located in any part of the digestive tract, but more frequently in the esophagus, duodenum, and small intestine. These ulcerations sometimes develop into pseudodiverticulae.

Aged↗
PubMed1980

[Elevation of unsaturated transcobalamin II in autoimmune diseases: study of the process in lupus erythematosis and dermatomyositis].

In an earlier study concerning patients with autoimmune diseases, a more reliable correlation of clinical disease activity was found with raised concentrations of the serum protein transcobalamin II than with several tests now in common use (complement factor C3, antinuclear antibody titer and antinative DNA binding capacity). These findings were subsequently confirmed in long-term observations over 1 to 3 years: the present paper discusses 5 of 7 investigated cases (2 with SLE, 2 with dermatomyositis of changing activity, and 1 with an erroneous diagnosis of SLE) in detail.

Adolescent↗
PubMed1980

Epidemiology of polymyositis-dermatomyositis in Israel, 1960-76.

We attempted to identify all cases with polymyositis and/or dermatomyositis (PM-DM) admitted to hospitals in Israel during 1960-76. The diagnosis of PM-DM was retrospectively confirmed in 86 Jewish patients. The age-adjusted incidence rates increased from 1.30 in 1960-64 to 2.57 in 1970-74, with an overall incidence of 2.18 new cases diagnosed annually per 10(6) population. The male:female ratio in this series was 0.59. Females predominated among patients younger than 60 years of age, while males and females aged 60 or more appeared to be equally affected. The age distribution was heterogeneous with modes in the second and seventh decades. The annual incidence of PM-DM increased from 0.47 cases per 10(6) persons in the third decade to a peak of 6.32 in the seventh. The incidence of childhood PM-DM among non-Western Jews(of African or Asian origin) was significantly higher than that among Western Jews (of European or American origin). On the other hand, the incidence of adult PM-DM among Western Jews exceeded that among Jews of non-Western extraction.

Adolescent↗
PubMed1980

Total creatinine kinase and isoenzyme MB activity in serum and skeletal muscle of a patient with dermatomyositis.

Total creatine activity was greatly increased, and creatine kinase MB isoenzyme was measurable in the serum of a patient with dermatomyositis. In contrast, total CK activity in an extract of skeletal muscle from this patient was markedly decreased as compared with normal muscle extracts, but contained about the normal proportion of the MB isoenzymes, 1.5%. We believe skeletal muscle to be the source of the MB isoenzyme in the serum, because the patient's electrocardiographic pattern was normal and she had no signs or symptoms of myocardial infarction.

Clinical Enzyme Tests↗
PubMed1980

Polymyositis-dermatomyositis: diagnostic and prognostic significance of muscle alkaline phosphatase.

The distribution and intensity of alkaline phosphatase deposition in 54 patients with dermatomyositis-polymyositis (PM-DM) was analyzed by the enzyme histochemical method. Increased enzyme reactivity of endomysial capillaries was found in 28% of patients, equally distributed between adult onset PM (Group I) and PM-DM with overlap in other connective tissue diseases (Group V). Patients with high endomysial capillary reactivity (R1 larger than or equal to 60) responded poorly to steroids, had an increased incidence of rheumatoid factor, and had less fiber degeneration/necrosis in their biopsies. Twenty-two percent of patients demonstrated prominent perimysial phosphatase reactivity localized in newly formed collagen and fibroblasts. Thirty patients (55%) demonstrated significant numbers of alkaline-phosphatase-positive fibers positively correlated with increased fiber degeneration/necrosis, endomysial fibrosis, increased numbers of triglyceride-containing muscle fibers, and NADH tetrazolium reductase hyperreactivity. Minimal overlap between the three enzyme distribution patterns was found. Endomysial capillary activity probably represents endothelial alkaline phosphatase induction analogous to the pattern seen normally in lower mammals (rat, rabbit, guinea pig). Alkaline phosphatase fiber reactivity probably represents a particular phase in fiber regeneration/maturation especially after denervation and is positively correlated with an increased incidence of spontaneous fibrillation potentials in PM-DM.

Adult↗
PubMed1995

[Dermatomyositis: successful therapy with high dosage intravenous 7S immunoglobulins].

This report describes the successful administration of high dose, intravenous 7S immunoglobulins (IG) to an 11 year-old girl with corticosteroid-resistant dermatomyositis. Clinical symptoms improved immediately and the pathological laboratory findings normalized within 10 days of commencement of immunoglobulin therapy. After three cycles of IG the patient showed complete remission of the muscular and dermatological findings. On follow-up examination after one year the patient remained symptom-free in the absence of any therapy and was leading a normally active life. The efficacy of IG therapy in this case is reviewed in the light of the literature.

Child↗
PubMed1995

Elevated cancer incidence in patients with dermatomyositis: a population based study.

OBJECTIVE: To study the relationship of polymositis (PM) and dermatomyositis (DM) and cancer in Finland. METHODS: The medical records of 175 patients with PM and 71 with DM diagnosed between 1969 and 1985 were reviewed. The patients were followed until death or until December 30, 1990 through the files of Statistics Finland and the Finnish Cancer Registry. RESULTS: Thirty-four cases of cancer were found [standardized incidence ratio (SIR) 2.1, 95% confidence interval 1.4-2.9]. In the PM group the SIR was 1.0 (95% CI 0.5-1.8), and in the DM group 6.5 (95% CI 3.9-10). The relative risk of cancer among DM cases was very high (SIR 26, 12-48) in the first year after DM diagnosis. CONCLUSION: DM is associated with cancer, but not PM. The risk is high among patients with DM older than 49. Cytotoxic drugs given to DM patients did not increase the risk of malignancies. DM and certain cancers seem to share etiological factors.

Adolescent↗
PubMed1995

Polyneuropathy in juvenile dermatomyositis.

We describe 2 patients in whom juvenile dermatomyositis (DM) was associated with well defined clinical polyneuropathies, and review the clinical and serological data. Light and electron microscopy were used to study muscle and nerve tissues from one patient. Neuropathy in our patients was associated with ulcerative skin lesions and elevated serum levels of factor VIII related antigen. Light microscopic studies of muscle revealed perifascicular atrophy and microinfarcts consistent with juvenile DM. Light microscopy of the affected sural nerve showed axonal degeneration. Electron microscopy of the same nerve demonstrated capillary endothelial inclusions characteristic of those observed as manifestations of early endothelial injury in juvenile DM muscle tissue. Polyneuropathy in patients with juvenile DM is a rare complication and is likely due to ischemia secondary to endothelial damage.

Biopsy↗
PubMed1995

[Muscle histopathology and responsiveness to steroid therapy in polymyositis and dermatomyositis].

We carried out muscle biopsies on 22 patients with polymyositis and dermatomyositis, and evaluated the relationship between muscle histopathological findings and steroid responsiveness. We classified the histopathological findings of the biopsied muscles into 4 types based on the distribution and extent of inflammatory cellular infiltration. The first "perivascular infiltration" type was seen in 7 patients who had inflammatory cells mainly around the small vessels, the second "endomysial infiltration" type in 5 patients who had inflammatory cells mainly around the muscle fibers, the third "diffuse infiltration" type in 4 patients who had scattered inflammatory cells around the small vessels and the muscle fibers, and the fourth "scant infiltration" type in 6 patients who had no prominent inflammatory cells. Most of the patients with the first or the second type showed a good response to the initial steroid therapy, while 3 patients with the diffuse infiltration and 4 with the scant infiltration types poorly responded. Histopathological typing of biopsied muscles is one of the useful markers to predict the responsiveness to the initial steroid therapy.

Administration, Oral↗