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PubMed2002

[Arrector pili myositis in a patient with dermatomyositis: an unusual histological feature].

Dermatomyositis is a rare inflammatory disease with a difficult diagnosis on cutaneous biopsies, since alterations are often little marked and non specific. A 52-year-old patient had severe paraneoplastic dermatomyositis. He had an erythematous rash on the trunk, and erythematous papules on the back of the hands. The first histological cutaneous lesion was a striking arrector pili myositis. Arrector pili myositis has rarely been described, and is thought to be linked with Wong type eruption occurring in dermatomyositis. Arrector pili myositis might constitute an helpful sign in the diagnosis of dermatomyositis, but its specificity and sensitivity are unknown. We compared this case to four previously reported cases. In these five cases, arrector pili myositis was not associated with a particular sub-type of dermatomyositis or with poor prognosis.

Biopsy↗
PubMed2003

[Erythroderma and multiple cutaneous necrosis revealing a dermatomyositis].

INTRODUCTION: We report an original case of dermatomyositis associated with neoplasia, which initial clinical expression was erythroderma and multiple cutaneous necrosis. OBSERVATION: A 64-year-old patient was admitted at hospital for erythroderma. He had a diffuse and inflammatory erythema with thrill, periorbital oedema, periungueal telangiectasia and epidermal necrosis. Physical examination also revealed symmetric proximal muscle weakness as well as hepatomegaly. There were biological signs of myolysis. Complementary investigations revealed a liver carcinoma with lung metastasis. The patient first underwent topical corticosteroid treatment, which provided partial improvement of the clinical and biological signs of disease. Thereafter he was treated with prednisone and tamoxifen. Death occurred at home 4 months after the diagnosis. DISCUSSION: Diagnosis of dermatomyositis was definitely set according to the criteria of Bohan and Peter. Epidermal necrosis, present in our observation, occur classically in dermatomyositis where they are a predictive factor of association with neoplasia. Other recognized predictive factors are the age of the patient and persistent itching. Erythroderma linked to dermatomyositis is a very unusual event: 4 cases have been reported in the literature, only one of which was associated with cancer. On the other hand, numerous cases of diffuse erythema were reported, which are close to erythroderma. It is not possible to set out that this clinical form is a factor of bad prognosis of dermatomyositis. As a matter of fact, in the published cases, one does not find more frequent deceases, pejorative signs or increased frequency of association with neoplasia.

Dermatitis, Exfoliative↗
PubMed2003

[Clinical characteristics of juvenile dermatomyositis].

INTRODUCTION: Dermatomyositis, belonging to the group of the idiopathic inflammatory myopathies, is characterized by bimodal pattern of age-specific incidence of rates, with peaks in age group from 5 to 14 years (juvenile dermatomyositis) and in age group from 45 to 64 years (adult dermatomyositis). THE AIM OF THIS STUDY: Is to evaluate the clinical characteristics of 12 patients with juvenile dermatomyositis followed by the 3rd Department of Internal Medicine, University of Debrecen and the 2nd Department of Pediatrics, Semmelweis University, Budapest. METHODS: The authors analyzed the medical records of the patients with juvenile and adult form of dermatomyositis retrospectively. RESULTS: All of the children had symmetrical weakness of the proximal muscles. The most frequent cutaneous features were facial erythema and Gottron papules (11/12). The extramuscular manifestations were also assessed. 7 children had arthralgia. There were observed pulmonary fibrosis, Raynaud-syndrome, dysphagia and sicca-syndrome in the same patient, whose disease is overlapped with progressive systemic sclerosis. In view of the clinical course, the authors found that prevalence of polycyclic (relapsing-remitting) and monophasic subtypes of the disease were similar (6/12 and 5/12). Finally, all of the patients achieved remission, however, 2 patients have to take low-dose corticosteroid therapy permanently to maintain remission. One patient's cutaneous symptoms proved to be persistent and in further 2 cases, regression of the calcinosis is slow, but continuous. DISCUSSION: The authors compare their data of juvenile patients with the data of the relevant literature and to their experience with the management of adult DM patients. It seems to be reasonable to treat the patients in centres.

Adolescent↗
PubMed2004

Chronic hepatitis C virus infection associated with dermatomyositis and hepatocellular carcinoma.

Dermatomyositis is a rare and idiopathic inflammatory myopathy with characteristic cutaneous manifestations. In recent years, some researchers have showed the cause of dermatomyositis might be due to an autoimmune response induced by viral infections. However, chronic hepatitis C virus (HCV) infection associated with dermatomyositis is very rare. In this report, we present a patient with dematomyositis with abnormal liver function test results and elevated alfa-fetoprotein level. After excluding multiple viral infections known to cause myositis, the case was proven to be chronic hepatitis C by positive HCV-RNA in the serum. Abdominal computed tomography showed a liver tumor on the right lobe and needle biopsy proved it to be hepatocellular carcinoma. Chronic hepatitis C or hepatocellular carcinoma might cause dermatomyositis by inducing the formation of autoantibodies. Chronic hepatitis C or hepatocellular carcinoma should be considered in patients of dermatomyositis if no other cause is found.

Aged↗
PubMed2005

[The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'].

This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a skin biopsy is advisable. A muscle biopsy is indicated when other examinations are inconclusive and the musculature is involved. The working group considers screening for cancer to be required in adults with dermatomyositis and presents recommendations for the way that this should be done. At least one-third of all patients with polymyositis has, or will develop, an associated inflammatory connective tissue disease. If a patient with a connective tissue disease develops symmetrical, proximal muscle weakness in the course of weeks or months, this may be assumed to be due to polymyositis. In the absence ofpre-existing connective tissue disease, demonstration of a mononuclear cell infiltrate in muscle tissue is a prerequisite for the diagnosis ofpolymyositis. The histopathology of muscle tissue is used as the gold standard for the diagnosis of sIBM. The practice guideline presents criteria for the concept 'activity' of myositis. Disease activity serves as a guideline for the treatment of polymyositis and dermatomyositis. The treatment of choice for dermatomyositis and polymyositis is high-dose prednisone. Physical activity does not have a negative effect on the course of these diseases. The long-term prognosis ofdermatomyositis and polymyositis is not well known. The clinical course of sIBM is slowly progressive.

Anti-Inflammatory Agents↗
PubMed2006

[Immunopathological changes of micro-vessels in dermatomyositis].

OBJECTIVE: To investigate the immunopathological changes of micro-vessels in dermatomyositis. METHODS: Twelve patients with dermatomyositis, 5 males and 7 females, aged 40.8 (6 - 72), underwent muscle biopsy of biceps muscle of arm (n = 11) or deltoid muscle (n = 1). The specimens underwent routine histological, enzyme histochemical, and immunohistochemical staining and microscopy. Ten biopsy specimens from patients with other diseases were used as controls. RESULTS: Perifascicular atrophy of muscular fibers and inflammatory infiltration in perimysium were seen in all patients with dermatomyositis. Non-specific esterase staining showed deep staining of capillaries and micro vascular endothelium among the muscular fibers. Immunohistochemistry showed remarkable reduction of capillaries positive in von Willebrand factor (vWF), thrombomodulin (TM), and endothelial cell nitric oxide synthase (eNOS) in the perifascicular region, and low expression of .eNOS and TM in the microvascular endothelium in the perimysium. CONCLUSION: The vascular lesions of dermatomyositis are located not only in capillaries, but also in other microvessels. Lower expression of TM and eNOS in vascular endothelium suggests the reduction of anticoagulation and vasodilation functions of vascular endothelium. Dermatomyositis is an inflammatory vascular endothelial disease.

Adolescent↗
PubMed1991

Peculiarities of dermatomyositis (DM) in early age.

The peculiarities of beginning of disease, clinical factors of risk in 70 children (aged under 8) with dermatomyositis are discussed. We have followed up 70 children (40 girls and 30 boys) with dermatomyositis at the age of 14 months to the age of 8 years. Most of the children were born in normal time, they have normal life functions during the first year of life. The most frequent intercurrent diseases were respiratory infections, angina; the chicken-pox was frequent too. 12 children had food allergy and 20 children-drug allergy. The most frequent factors preceded dermatomyositis were respiratory diseases and inoculations. Assembling of genealogical anamnesis was made according to "family portrait". 14 families of children with dermatomyositis (369 relations in I-IV degree of relationship) were examined. Genealogical investigations revealed the high frequency of rheumatological pathology in proband's families. Small anomaly of development among probands (M +/- m = 12.5 +/- 0.6) exceeded these quantity comparing to the control group (n = 60). Dermatollphyics (98 indices on each child) contained a number of peculiarities for forming the group of risk. Onset of disease was acute or subacute in 2/3 of children. Primary chronic onset and the progress of the disease took place in 10 cases. An acute onset of dermatomyositis was characterized by fever, myaglia, arthralgia, bright skin symptoms: widespread purple violet face erythema, a "butterfly wing" or "paraorbital glasses", palmal erythema and widespread vascular manifestations on face, chest, back and limbs in the form of net--"livedo reticularis".

Child↗
PubMed1989

[Neuromuscular blockade using vecuronium in dermatomyositis].

The significant features of neuromuscular blockade with vecuronium in a patient with dermatomyositis are described: vecuronium 0.08 mg/kg resulted in 90%, 0.12 mg/kg in 100% neuromuscular blockade. In contrast to claims made in some previous publications, dermatomyositis did not produce increased sensitivity to vecuronium. Onset time and duration of action were also within normal limits in our patient. Time of spontaneous recovery until antagonism with neostigmine was markedly prolonged, but the dermatomyositis was only one of various possible explanations. Although there are potential hazards in the use of neostigmine in patients with dermatomyositis, antagonism of the neuromuscular block with 2 mg neostigmine was without problems in our patient. Our data support recent suggestions to reconsider the implications of dermatomyositis for anesthesia.

Aged↗
PubMed1983

[Dermatomyositis and colonic cancer. Review of the literature apropos of a case].

The authors report the case of a 56 year-old woman presenting with dermatomyositis revealing a cancer of the colon at the ganglial dissemination stage. Exeresis led to the regression of the dermatomyositis and allowed an attenuation of the steroid therapy. The muscular and cutaneous signs reappeared within six months, evidencing a metastatic hepatic dissemination. Death came within six months of the appearance of the dermatomyositis. The authors review the literature concerning dermatomyositis associated with cancers of the digestive tract in general, and of the colon in particular. This association is found in about 6 to 35% of cases of dermatomyositis, which is the most common paraneoplastic skin associated with cancers of the colon.

Colonic Neoplasms↗
PubMed1982

[Pulmonary lesions in dermatomyositis. A retrospective study of 4 cases].

During a retrospective study of the records of 9 patients with dermatomyositis, pulmonary lesions were noted in 4 cases. Dermatomyositis had been diagnosed on clinical, biochemical, electrical and histopathological criteria. Patients with other connective tissue disease associated with dermatomyositis were excluded. In one case the pulmonary lesions preceded the clinical symptoms of dermatomyositis. Two patients had interstitial pneumonia and 2 presented with reticulonodular images suggestive of diffuse interstitial fibrosis. The outcome after corticosteroid and/or immunosuppressive therapy was favourable in 3 cases. It would appear from this study that clinical and radiological signs of pulmonary lesions and respiratory insufficiency are abnormally frequent in dermatomyositis and that the prognosis in such cases is perhaps brighter than is usually believed.

Adult↗
PubMed1994

[A clinical analysis of cutaneous type dermatomyositis].

This paper reports nine patients with the classic cutaneous findings of dermatomyositis who did not develop clinical or laboratory evidence of muscle disease for at least 2 years after onset of their skin manifestations. Such patients represent 3.5% of our total experience with dermatomyositis patients during a 12 years period. None of the patients had evidence of malignancy. Each of five patients treated with oral prednisone for their cutaneous lesion or mild myositis after onset of their skin manifestations 3-12 years and had marked improvement. The author emphasizes that the cutaneous manifestations of dermatomyositis are pathognomonic of this disease and the term of this disease proposes cutaneous type dermatomyositis better than amyopathic dermatomyositis.

Adult↗
PubMed1994

[Dermatomyositis and cancer. A retrospective study].

In a retrospective study, the files of 19 patients with dermatomyositis examined at our departments from 1970 to 1993 were reviewed. The parameters studied were age, sex, muscle enzyme values, muscle biopsies, electromyographical findings and interval from onset of dermatomyositis until first visit to the department. Out of 19 patients with dermatomyositis, 18 were adults and in nine of these the condition was associated with cancer (three out of three men, six out of 15 women). Electromyographical findings were pathological in 17 investigated patients and myositis was indicated in 13 out of 15 biopsies. Muscle enzyme values were elevated in seven out of nine patients with cancer and in three out of nine without. Out of five patients with dysphagia, four patients had cancer. The risk of cancer is increased in patients with dermatomyositis. Factors indicating a poor prognosis regarding the association between dermatomyositis and cancer in our study were old age, male sex and dysphagia.

Adult↗
PubMed1997

[Prognostic factors of dermatomyositis: analysis of 119 cases].

In order to know the prognosis of dermatomyositis, 119 patients with dermatomyositis were investigated from June to September 1995 by Shanghai Medical Cooperation Center on Dermatomyositis. Ten prognostic factors were studied with Lifereg Procedure multiple factor analysis. The results showed that 2 and 5-year survival rates were 75.3% and 53.2% respectively. The most common cause of death was lung infection and then was malignant tumor. Unfavorable prognostic factors were old age, malignant tumor, interstitial lung disease, dysphagia, cardiac involvement, fever, failure of remission and steroid therapy alone. The survival curve showed that mortality rate was high in the first three years, less between three and seven years and none after seven years. The results showed that dermatomyositis is a disease with poor prognosis. Patients with dermatomyositis can be adversely affected by many factors and should be treated regularly with steroid and immunosuppresive drugs as early as possible.

Adolescent↗
PubMed1994

Dermatomyositis with normal muscle enzyme concentrations. A single-blind study of the diagnostic value of magnetic resonance imaging and ultrasound.

BACKGROUND AND METHODS: It is well documented that the cutaneous lesions of dermatomyositis may precede clinical myositis or may occur in the absence of any muscle disease detectable by current diagnostic criteria. In this single-blind study, we used magnetic resonance imaging (MRI) and ultrasound to evaluate five patients who presented with classical clinicopathologic dermatomyositis, but with normal levels of serum muscle enzymes. This patients group has not been previously studied with these techniques. Patients who served as positive and negative control subjects were also examined. RESULTS: Ultrasonography revealed hyperechogenicity, and MRI revealed high signals on T2-weighted images in several muscle groups of the patient with active myositis (positive control). Increased echogenicity was also noted in the deltoid region of one patient who had previously had a normal muscle biopsy finding. In the same patient, MRI revealed inflammatory changes in the lumbar paraspinal muscles. Another patient, with all previous study results being normal, had MRI evidence of T2 high signals in the gluteus minimus. CONCLUSIONS: Noninvasive examinations such as MRI and ultrasound are beneficial as adjunctive means of examination in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. Future studies may suggest additional uses for these tests, including serial evaluation of patients, noninvasive confirmation of diagnosis in pediatric patients, or in directing muscle biopsy, thus increasing sensitivity. Ultrasound appears to be the more cost-effective and simple test; but MRI, although more expensive, may be more sensitive and specific.

Adult↗
PubMed1981

Patients with polymyositis and dermatomyositis who undergo plasmapheresis therapy. Pathologic findings.

Muscle biopsy specimens were studied in 20 of 26 patients who had polymyositis or dermatomyositis prior to treatment with plasmapheresis and immunosuppressive therapy. Morphologic findings from biopsy specimens were similar among patients with polymyositis and dermatomyositis, except that perifascicular atrophy was a prominent feature in patients with dermatomyositis and inconspicuous or absent in patients with polymyositis; immunofluorescent staining of muscle for immunoglobulins and/or complement was noted more frequently for patients with dermatomyositis than those with polymyositis. Patients judged clinically to have highly active disease had the most severe changes, which consisted of muscle necrosis, regeneration, phagocytosis, and inflammation; those with mild and moderately active disease were indistinguishable pathologically. No correlation between duration of disease and degree of pathologic alteration was found. Six of seven patients with both pretreatment and posttreatment biopsies showed marked improvement in the extent of pathologic alteration, and a statistically significant reduction in the degree of muscle atrophy following treatment.

Adolescent↗
PubMed1991

The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis.

Accumulating evidence indicates that a complement-mediated microvasculopathy may play a pathogenic role in dermatomyositis. In a previous study, we demonstrated neoantigens of the C5b-9 complement membrane attack complex in the muscle microvasculature of childhood and adult cases of dermatomyositis. To further characterize the relationship between the vascular complement deposits and histologic changes, quantitative histopathologic analyses were performed on 39 dermatomyositis biopsy specimens (26 adult, 13 children). There was a significant correlation between the percentage of fascicles with fibers having focal myofibrillar loss, a change seen early in the evolution of ischemic muscle fiber damage, and the percentage of fascicles having capillary deposits of membrane attack complex. Conversely, in biopsy specimens with a higher percentage of fascicles with perifascicular atrophy, membrane attack complex deposits were significantly less common. A fascicle-by-fascicle analysis supported these observations. Patients whose biopsy specimens were negative for microvascular membrane attack complex had clinical weakness for a significantly longer time than those patients with vascular complement deposits. These data support the hypothesis that the complement-mediated vasculopathy is a primary immunopathogenic event in the evolution of muscle lesions in dermatomyositis.

Adult↗
PubMed1990

Microvascular changes in early and advanced dermatomyositis: a quantitative study.

In adult dermatomyositis 10 muscle specimens with no or minimal histological alterations were compared with 7 that showed typical alterations. Five specimens from patients with inclusion body myositis, 5 from patients with polymyositis, and 8 from normal subjects served as controls. Vascular endothelium, visualized with the lectin Ulex europaeus agglutinin I, and complement membrane attack complex were demonstrated in the same cryostat sections by paired immunofluorescence. Large randomly selected fields were analyzed to determine the number of capillaries per square millimeter of fiber area (capillary density), per 1,000-microns 2 area of each muscle fiber (capillary index), and in 100 x 100-microns grid squares. In dermatomyositis specimens with minimal structural alterations there was focal capillary depletion, the capillary density was significantly reduced, and the frequency distributions of the capillary index and grid count were shifted to the left. In advanced dermatomyositis specimens, the findings were similar but more severe. In both kinds of specimens, clusters of capillaries reacted for complement membrane attack complex. The 2 patients with the highest proportion of vessels positive for membrane attack complex had a fulminant and fatal course. In polymyositis and inclusion body myositis specimens, the capillaries had a normal overall density and none reacted for membrane attack complex. The findings imply that the capillaries are an early and specific target of the disease process in dermatomyositis.

Adult↗
PubMed1982

Gamma-carboxyglutamate excretion and calcinosis in juvenile dermatomyositis.

Proteins containing gamma-carboxyglutamic acid (Gla) are present in subcutaneous calcifications of adults with dermatomyositis or scleroderma. Sixteen children with juvenile dermatomyositis, including 7 with subcutaneous calcifications, were studied to determine if abnormal synthesis or turnover of Gla-containing proteins occurred. All study children had increased excretion of the amino acid that was greater than that of age- and sex-matched controls. Patients who had juvenile dermatomyositis with calcifications had a 3-fold increase in Gla excretion, and those without calcinosis had a 2-fold increase. Five other children with various connective tissue disorders and subcutaneous calcification had 2-fold increased Gla excretion. Decreased excretion of this amino acid was associated with salicylate therapy (80 mg/kg/24 hours). The data suggest an abnormal turnover of Gla-containing proteins in juvenile dermatomyositis. Metabolism of these proteins may be involved in the pathophysiology of soft-tissue calcification in children.

1-Carboxyglutamic Acid↗