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Results for “DERMATOMYOSITIS”

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PubMedDate not supplied

[Study of the immune system in relatives of children with dermatomyositis].

In view of the fact that relatives of patient with other connective tissue diseases may show immunologic abnormalities, we investigated cellular (PPD, SK-SD, candidine and diphteria-tetanous toxoid cutaneous tests) and humoral (IgG, IgM, IgA. CH50, C3, rheumatoid factor and ANA) immune responses in first degrees relatives (parents and siblings) of children with dermatomyositis. Of the 53 subjects tested, 19 had high values of one or two immunoglobulines; however, the mean Ig value was not different from that of the general population (p greater than 0.1), CH50, C3, rheumatoid factor and ANA were normal or negative. Cutaneous tests made on 46 subjects did not reveal abnormalities. Therefore our conclusion is that first degree relatives of children with dermatomyositis have not significant immunologic abnormalities.

Adolescent↗
PubMed1979

Dermatomyositis and female malignancy.

All cases of dermatomyositis-polymyositis in women seen between 1970 and 1977 at Baylor Affiliated Hospitals were reviewed. Twenty-five patients were identified with this diagnosis. Of these women, 5 were found to have a malignant tumor: 3 ovarian carcinomas, 1 cervical intraepithelial carcinoma, and 1 colon carcinoma. Pertinent histologic and clinical findings in patients with dermatomyositis-polymyositis are discussed. The high prevalence of malignancy in these patients, previously recognized, is discussed. A case report is presented.

Adult↗
PubMed1979

Dermatomyositis-like syndrome due to toxoplasma.

A woman had the typical clinical, cutaneous, and muscular picture of dermatomyositis but without some of the biochemical alterations of that disease. The positive serological investigations for toxoplasmosis, the pressure in muscles of very numerous toxoplasms and their culture in mice, and the improvement after treatment with pyrimethamine and sulphametoxypyridine demonstrated that the signs of dermatomyositis had been caused by infection with toxoplasmosis.

Adult↗
PubMedDate not supplied

[Calcinosis universalis associated with dermatomyositis. A report on five cases (author's transl)].

Five patients with dermatomyositis developed calcinosis universalis, the calcinosis lesions being diffuse in three cases and localized to a subcutaneous site in the other two. The diffuse lesions developed in young subjects with severe dermatomyositis, and increased progressively during recovery from the muscle disease. Treatment was ineffective. Subcutaneous calcifications can be detected at an early stage by technetium pyrophosphate scintigraphy, while the fluid nature of the swellings due to a local inflammatory reaction is proved by ultrasonography. Good results are obtained after excision of those calcified masses requiring removal because of their size, site, or superinfection.

Adolescent↗
PubMedDate not supplied

[Dermatomyositis in pediatrics].

The study included 19 children with dermatomyositis. All showed frequently atypical cutaneous disords which in most cases were the initial manifestation. In every case there was some clinical evidence of proximal myopathy. Arthralgias were frequently found and sinusal tachycardia was present in 10 cases. The correct diagnosis was made from the beginning only in 6 cases. Systemic lupus erythematosus was the main cause of confusion. The differential diagnosis between these two entities cannot be made only by the presence or not of nephropathy, even if this disease was evident in 42% of patients with dermatomyositis. The most useful laboratory tests for the diagnosis and management were CPK, aldose and serum TGO. Electromyography was abnormal in every case where it was practiced. Muscular biopsy was valuable in 15 of the 19 patients. All were treated with prednisone and in most cases with good results.

Adolescent↗
PubMed1984

[Pediatric dermatomyositis. Apropos of 28 cases].

The authors report 28 cases of juvenile dermatomyositis. Bohan and Peter criteria were fulfilled in all cases. All children received a treatment with prednisone as soon as diagnosis was established. Five patients (18%) died during this treatment. The remaining children were followed for at least 2 years after prednisone treatment was discontinued. An acute onset was found in 4 of the 5 children who died and in 6 of 23 children still alive. Swallowing disorders were present in all children who died and in 11 of the children still alive. Ten children recovered fully and 13 presented with sequellae. The average interval between clinical onset and diagnosis was 2 months 19 days for the children who recovered and 5 months 21 days for those presenting with sequellae. The average duration of the period of activity of dermatomyositis was 6 months for the children who recovered and 3 years 4 months for those with sequellae.

Adolescent↗
PubMedDate not supplied

[Electromyographic findings in children with dermatomyositis].

The author analysed electromyographic records in 14 children wit dermatomyositis. The findings included primary muscular changes of great intensity and presence of activity at rest in 50% of cases. The intensity of primary muscular changes resembled that observed in Duchenne's dystrophy and the distribution of changes was similar to that in non-progressive myopathies. Electromyographic changes occur in all cases of dermatomyositis and confirm the clinical diagnosis.

Action Potentials↗
PubMedDate not supplied

[Combination of ankylosing spondylitis with dermatomyositis and atypical gout (isolated gout kidney)].

In a 58-year-old man with the clinical and radiological signs of definite ankylosing spondylitis in its final stages, HLA B 27 positive, an onset of dermatomyositis was observed with characteristic clinical, histological, and pathological findings, and with high titers of ANA. It had been discovered years before that a gouty kidney with pyelonephritis accompanied the advanced ankylosing spondylitis. On the basis of these findings a triple association of etiologically different diseases could be diagnosed. After treatment with cortisone, allopurinol, and azathioprine appeared an acute and no longer controllable panmyelophthisis. That a malignant tumor could have caused the dermatomyositis was excluded by autopsy. Diagnostic questions concerning the gouty kidney as well as factors explaining the acute and deadly progress of a panmyelophthisis after the administration of a rather mild immunosuppressive agent are discussed.

Dermatomyositis↗
PubMed1983

Dermatomyositis in a skin clinic.

12 cases of dermatomyositis were seen in the Middle Road Hospital over a five year period. 2 cases were childhood dermatomyositis. Of the 10 adult patients, the majority (7) were in the younger age group between 21 to 40 years old. The incidence of underlying malignancy was 60% in adult patients and of all the malignancies, 66.7% were nasopharyngeal carcinoma. 4 of the 6 patients with underlying malignancy were in the younger age group of 21-40 years old which was previously reported as uncommon.

Adult↗
PubMed1981

Ultrastructural abnormalities and perifascicular atrophy in childhood dermatomyositis with special reference to transverse tubular system-sarcoplasmic reticulum junctions.

Comparison of ultrastructural abnormalities between perifascicular and centrofascicular myofibers was made in the same muscle fascicles from six patients with childhood dermatomyositis. Consistent abnormalities are seen at the junctional sites between the transverse tubular system (TS) and sarcoplasmic reticulum (SR) as visualized by lanthanum staining. Early conical dilations at the TS-SR junctions have progressed to become connected with large cylindrical or spheroid channels representing SR. These TS-SR anastomoses are far more extensive in the perifascicular than in the centrofascicular myofibers. Honeycomb TS proliferations suggestive of a repair process occasionally occur at the sites of TS-SR anastomoses. These alterations precede myofibrillar derangements and they may be related to the primary pathogenetic process involved in dermatomyositis. It is postulated that leakage of degraded structural protein molecules through TS-SR anastomoses may lead to the perifascicular atrophy seen on light microscopy.

Adolescent↗
PubMed1982

Dermatomyositis and dermatitis herpetiformis.

Primary hypothyroidism, dermatomyositis, dermatitis herpetiformis (DH), and Sjögren's syndrome developed sequentially, over a 20-year period, in a 67-year-old woman. The principal manifestations of her illness were periodic and simultaneous exacerbations of dermatomyositis and DH. Her histocompatability antigens were A1,B8, a haplotype associated with autoimmune diseases. We suggest that this unusual disease complex has a genetic basis.

Aged↗
PubMed1982

[Epidemiology of connective tissue diseases of the skin (scleroderma, dermatomyositis and polymyositis) in Japan].

An epidemiologic study of connective tissue diseases (scleroderma, dermatomyositis, and polymyositis) performed from 1973 to 1976 in Japanese university hospitals is reported. Data from 32 of 48 university hospitals revealed that a total of 659 cases were collected. There were 396 cases of progressive systemic sclerosis, 178 cases of dermatomyositis, and 85 cases of polymyositis. The following points were analyzed: relationship of regional incidence, sex, initial symptoms, laboratory findings, and statistical analysis of autopsies.

Adolescent↗
PubMed1981

[Dermatomyositis associated with lethal pulmonary fibrosis and cutaneous necrosis (author's transl)].

This clinical report concerns a 57 year old woman with dermatomyositis and cardiac involvement. After an apparent clinical and biological improve of the muscular and myocardic symptoms, a pulmonary involvement was reported. There was a persistent high rate of lacticodeshydrogenase serum activity. Then, cutaneous ulcerations and necrosis appeared on the extremities of limbs. Dermatomyositis associated with cutaneous necrosis and pulmonary fibrosis has a very severe prognosis.

Dermatomyositis↗
PubMed1981

[A case report of a primary pure squamous cell carcinoma of the stomach associated with dermatomyositis (author's transl)].

A 54-yr-old man was admitted to Hokkaido University Hospital, complaining of fever, multiple arthralgia, edematous erythema and face and muscular weakness of extremities during the last 2 months. He was diagnosed as dermatomyositis by acceleration of ESR, elevation of GOT, GPT, CPK, aldolase, moderate increases of collagen fibers in biopsy specimen of skin and his clinical signs. Although stools were positive for occult blood, the routine radiographic examination failed to detect the bleeding site in the upper GI. tract. However, in the double contrast picture of the stomach, a very fine abnormal linear shadow was observed at the upper corpus of the lesser curvature. This linear shadow was a margin of the tumor, retrospectively. About 4 months later, abnormal pain occurred and a mass was palpable in the left lumbar region, suggesting a pancreatic tumor. He was operated on excising the tumor, but was performed only exploratory laparotomy because of the presence of intra-abdominal metastases. Death occurred 40 days after the operation and necropsy was done. The gross anatomical findings of the abdomen showed a stomach tumor as large as an infant's head and its metastases to pancreas, lymph nodes, and greater and lesser omentum. Esophageal mucosa including esophagocardiac junction was intact. Histological examination of the intragastric tumor revealed a typical squamous cell carcinoma with keratinization. According to the absence of the components of adenocarcinoma and squamous metaplastic gastric mucosa of non-cancerous areas in the stomach, it seemed likely to be a heterotopic squamous cell carcinoma. It was unknown about the precedence between the stomach cancer and dermatomyositis. There have been 11 cases of primary pure squamous cell carcinoma in the world literature since 1968, but this is the first case report of coexistence of these two diseases.

Carcinoma, Squamous Cell↗
PubMed1981

Dermatomyositis: cutaneous clues to subclinical myositis.

A young woman presenting with Raynaud's vasospasm and polymorphic skin rash was found to have dermatomyositis. The myositis was subclinical at the time of her initial evaluation, although biochemical, electromyographic and histologic studies confirmed muscle involvement. Proper diagnosis and treatment of dermatomyositis requires recognition of the frequent disparity between cutaneous and myopathic symptoms.

Adult↗
PubMed1980

[Systemic lupus erythematosus and dermatomyositis in children].

The authors study the diagnosis and the prognosis in 25 children with systemic lupus erythematosus and in 22 children with dermatomyositis. The most frequent signs when the diagnosis is established are articular involvements, erythema of the face, renal disorders and the presence of LE cells. The symptoms are often misleading, which explains the long delay between the first sign of the disease and the time of diagnosis, which can vary between one month to nine years. The prognosis of this disease has improved because of modern treatments : only four patients died, three of them because of renal failure and one because of cerebral embolism, after six months, one year, three years and ten months, In dermatomyositis the most frequent signs at the time of diagnosis are skin involvements and muscular and articular symptoms. The period of time between the onset of the disease and the diagnosis is shorter : one month to four years, 19 times equal to or less than one year, because the clinical picture is quite clear. Ten times the clinical evolution lead to a complete cure, 9 times a clear improvement with sequelae was observed : retractile myelosclerosis, calcinosis and medullary compression. One patient died of intestinal ulceration and two of pneumonia aggravated by involvement of the respiratory muscles.

Adolescent↗