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Childhood dermatomyositis: serial microvascular studies.

Childhood dermatomyositis is an inflammatory condition affecting the skin and muscles that is often associated with a small vessel vasculopathy. According to previous retrospective studies, it is suggested that the severity of the vasculopathy is related to the course of the disease. Sequential in vivo nailfold microscopy was used to assess the frequency and the degree of vasculopathy in nine children with dermatomyositis. The degree of morphologic changes in the nailfold capillary bed was shown to correlate with the clinical course. The technique of nailfold capillaroscopy may prove to be a clinically useful, noninvasive means of early prognosis.

Capillaries↗

[Juvenile dermatomyositis. Clinical and epidemiologic experience].

27 cases with a diagnosis of Juvenile Dermatomyositis were studied from a total of 1307 patients who suffered from connective tissue diseases; 19 of which met the diagnosis criteria of Bohan and Peters. Females were the most frequently affected. 52.63% of the cases presented the first symptoms between 5 and 9 years of age. Skin lesions, debility in inferior extremities and fever were the most frequent motives of consultation. The Aldolase and LDH were the muscular enzymes whose values increased in the majority of the cases. The electromyography was more sensible than the muscular biopsy in the diagnosis of the disease. We suggest that the epidemiologic and clinical characteristics found in our study should be taken into account for further accurate diagnosis of Juvenile Dermatomyositis in Venezuela.

Adolescent↗

[Plasma exchange in dermatomyositis and polymyositis. Retrospective study of 38 cases of plasma exchange].

In order to evaluate the efficacity of plasma exchanges (PE) in dermatomyositis and polymyositis, the case histories of 38 patients, who had undergone plasma exchanges between 1980 and 1986 in 10 French plasmapheresis centers, were studied. Large volume PE were performed in 34 patients after failure of conventional therapy and were part of the initial regimen for the 4 others. The initial activity of the muscle disease was acute in 27 patients and subacute or chronic in 11 cases. Clinical results were evaluated on a functional scale based on changes in muscle force: 24 patients improved (10 appreciably and 14 moderately) and 14 remained unchanged. PE were well tolerated in 23 patients. But side effects occurred in 15 patients, necessitating treatment withdrawal in 4 cases. PE seem to be more effective when the dermatomyositis is acute and evolutive than when it is chronic and insidious. The encouraging results of this exhaustive retrospective study prompt us to set up a prospective randomized trial.

Adolescent↗

Staphylococcal pyomyositis with idiopathic dermatomyositis.

We have described a patient with staphylococcal pyomyositis of the left pectoralis major muscle, complicating idiopathic dermatomyositis. Dermatomyositis and corticosteroids are possible predisposing factors. Patients with idiopathic muscle inflammation should be considered at risk for bacterial superinfection.

Adolescent↗

Recurrent pneumomediastinum in adult dermatomyositis.

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.

Adult↗

[Recurrent juvenile pancreatitis in dermatomyositis].

The authors present the case history of an 11-year-old girl who suffered from the rarely occurring grave juvenile dermatomyositis associated with recurring pancreatitis. Factors which may cause pancreatitis: juvenile dermatomyositis as underlying disease, steroid-, cytostatic- and thiazide diuretic treatment are analysed.

Adrenal Cortex Hormones↗

[The association of dermatomyositis with cancer: myth or reality?].

We present the case of a 56-year-old woman with proven dermatomyositis associated with an adenocarcinoma of the gallbladder. The association of dermatomyositis-polymyositis and cancer has been known for many years but is still controverted. The search for a malignancy in a patient with DM-PM should not be systematic but guided by abnormal history, physical examination and laboratory results.

Adenocarcinoma↗

[The principle antinuclear and anti-cytoplasmic antibodies in polymyositis and dermatomyositis. Study of 42 cases].

Using indirect immunofluorescence methods on rat liver sections and HEp-2 cells, combined with immunoprecipitation in agar, we were able to detect antinuclear antibodies in 76% of sera from 42 patients with polymyositis or dermatomyositis. In addition, 7% of the patients had anti-cytoplasmic antibodies without antinuclear antibodies. With immunofluorescence, the most common pattern was a nuclear fluorescence that was homogeneous on rat liver sections (11 cases) and speckled on HEp-2 smears (16 cases). The frequency of antinuclear antibodies was the same in the different categories of myositis as classified by Pearson. Precipitating antibodies were observed in 15 cases (36%). Two specific antibodies were frequently encountered: anti-Jo1 in 8 cases (19%) and anti-Pm-Scl in 5 cases (12%). Anti-Jo1 antibodies were present in both polymyositis and dermatomyositis. Seven out of the 8 patients with anti-Jo1 antibodies had pulmonary fibrosis (P less than 0.03).

Antibodies↗

Methotrexate and azathioprine treatment of childhood dermatomyositis.

Modern treatment of childhood dermatomyositis with corticosteroids has resulted in greatly improved prognosis and style of life. The immunosuppressive drugs methotrexate and azathioprine have been utilized as ancillary agents in life-threatening disease and in children whose disease could not be adequately controlled with prednisone alone. Two patients are completely well without medication two and seven years after onset; two have received no medications for more than two years and have only subtle signs of any residual illness although they had been profoundly ill for two to three years after the onset of dermatomyositis. A fifth patient, completing the third year of disease, remains ill and continues to require medications but has improved sufficiently to return to regular class in school.

Azathioprine↗

Concurrence of ovarian cancer and dermatomyositis. A report of two cases and literature review.

The concurrence of dermatomyositis and ovarian carcinoma deserves special attention owing to the gravity of its prognosis. Until now, only scattered case reports on women suffering from this disease combination had been published. In this paper the information available in the literature on 28 women afflicted with these diseases is collated. In addition, two such patients treated in our hospital are described in detail. The review highlights the various aspects of this disease combination, emphasizing the quadrupled incidence, advanced stage and uniform epithelial origin of the malignancy as compared with that in the general female population with ovarian carcinoma. The value of a meticulous gynecologic evaluation was proven in one of our patients with dermatomyositis; in her, ovarian carcinoma was detected in its early phase.

Adult↗

Dermatomyositis and granulomatous myopathy associated with sarcoidosis.

A previously healthy, 21-year-old female presented with acute dermatomyositis. Chest X-ray revealed bilateral hilar adenopathy, and mediastinal lymph node biopsy demonstrated noncaseating granulomata compatible with sarcoidosis. The patient improved spontaneously. The significance of bilateral hilar adenopathy in association with dermatomyositis and implications for management are discussed.

Adult↗

Quantitative muscle testing in childhood dermatomyositis.

Sixteen children with dermatomyositis were followed longitudinally by quantitative muscle testing between 1972 and 1982. The time to achievement of normal muscle strength measured quantitatively was significantly delayed (p less than .001) compared to the time muscle strength was clinically assessed as normal by manual muscle testing. A significant difference was also found between the time normal muscle strength was measured and normal muscle enzymes were achieved (p less than .05). Quantitative muscle testing is a useful adjunct to clinical assessment of muscle strength and determination of muscle enzymes in the therapeutic management of patients with dermatomyositis.

Adolescent↗

Jaccoud's arthropathy in adult dermatomyositis.

A patient with dermatomyositis developed a non-erosive deforming arthropathy of the hands and feet, consistent with Jaccoud's arthropathy. This type of arthropathy has not been described in dermatomyositis or polymyositis.

Dermatomyositis↗

[Prognosis and current treatment of dermatomyositis].

The present revision has been carried out in order to final out to what extent corticosteroids have improved the vital and functional prognosis of dermatomyositis. Over the sixties and in the early seventies, statistical tables have not shown any substantial differences compared with those of the pre-steroid era. It has been observed since 1973 that early treatment with high doses (1-2 mg of prednisone per kg per day) clearly improves the vital prognosis of infantile dermatomyositis, but not the functional one. In adults, there is less experience and the problem always exists of a possible underlying neoplasm. In cases with resistance to corticosteroids, the association of immunosuppressors, particulary methotrexate, has begun to be tested. There is no proved effective treatment of calcinosis which results in functional recovery.

Adult↗

Calcinosis as the presenting sign of juvenile dermatomyositis in a 14-month-old boy.

We describe a 14-month-old child with dermatomyositis in whom calcinosis was the first sign of the disease. This case shows that calcinosis, usually a late complication of dermatomyositis, may be the presenting sign of the disease even in young children and when Gottron's sign is still absent and muscular weakness not prominent.

Calcinosis↗

Postmortem findings in four litters of dogs with familial canine dermatomyositis.

Postmortem evaluations were performed on 20 juvenile to young adult collie and collie-Labrador retriever crossbred dogs with dermatomyositis and 10 neonatal collies. Cutaneous, muscular, and vascular lesions were present in the juvenile and adult dogs and were most severe in areas of the head and distal extremities. In more severely affected dogs, lesions were more generalized, including myositis of esophageal muscle and arteritis of skin, muscle, bladder, and spermatic cord. Although viruses were not isolated from muscle, crystalline viral-like structures were present in cytoplasm of endothelial cells within skeletal muscle. The dogs with dermatitis and myositis consistently had lymphoid hyperplasia, especially of peripheral lymph nodes. More severely affected dogs were smaller than less severely affected littermates, and the more severely affected males had reduced weight of testicles and prostate glands, compared with body weight. The reduced weight of genital organs correlated positively with reduced fertility. A few lymphoid aggregates were present in or around thyroid glands of 6 of the 20 dogs. There was no histologic evidence of glomerular disease in any of the dogs. The neonatal collies had no evidence of dermatomyositis.

Animals↗

Dermatomyositis with prominent mucinous skin change. Histochemical and biochemical aspects of glycosaminoglycans.

Although there is occasionally remarkable accumulation of mucin in the skin lesion of dermatomyositis, no special attention has been paid in regard to mechanisms underlying it. We carried out histochemical and biochemical studies on materials obtained from one case of dermatomyositis, who showed the presence of abundant mucinous material stainable with alcian blue between the collagen bundles throughout the lesional dermis. The major component of the ground substance was identified to be hyaluronic acid. The activity of N-acetyl-beta-glucosaminidase, exoglycosidase that degrades hyaluronic acid in serum of the patient, was equivalent to that of controls. However, heparan sulfate was greatly increased in the urine of the patient in comparison with that of the normal controls in which the major components were chondroitin 4-sulfate and chondroitin 6-sulfate. We think that the increase of hyaluronic acid in the lesional dermis is due to an excess production by the fibroblasts under some immunological stimulation, although its relation to the increased heparan sulfate excretion into the urine of the patient is obscure.

Acetylglucosaminidase↗

Myocardial infarction in dermatomyositis.

A case of dermatomyositis manifesting electrocardiographic and vectorcardiographic changes compatible with an inferior and anterolateral wall myocardial infarction is presented herein. The nonocclusive nature of the heart muscle damage is evidenced by perfectly normal coronary artery angiography. We believe that this is the first reported case of a myocardial infarction pattern apparently due to dermatomyositis.

Adult↗