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At least 217 records · Page 12Linked to original sources
PubMed1984

Duodenal perforation in childhood dermatomyositis.

Perforation of the duodenum is an uncommon, but serious complication which may occur in children with dermatomyositis. In this disease vasculitis may involve the bowel to a variable extent and result in radiologic manifestations of intestinal injury ranging from benign pneumatosis intestinalis to signs of bowel perforation. We report two children with dermatomyositis in whom perforation of the second portion of the duodenum occurred. This serious complication should be considered in any child with dermatomyositis when extraluminal gas is suspected on abdominal radiographs.

Adolescent↗
PubMed2000

Dermatomyositis after interferon alpha treatment.

An association between auto-immune disorders and interferon (IFN) has been reported. High levels of natural IFNalpha are present in the blood of patients with auto-immune disease and correlate with disease activity. In addition, IFNalpha treatment of humans has resulted in multiple reports of associated auto-immune phenomena. We describe a patient who underwent resection of regionally metastatic melanoma, was given adjuvant high-dose IFNalpha2b, and subsequently developed dermatomyositis. To the authors' knowledge this is the first report of dermatomyositis in association with IFNalpha treatment. We review the literature reporting associations between IFNalpha and auto-immune disease and discuss possible mechanisms by which IFNalpha may contribute to the development of auto-immune disease. High dose IFNalpha2b is more commonly prescribed since it was approved as an adjuvant treatment for patients with surgically resected high-risk melanoma. The potential for cases of IFN-associated auto-immune disease is therefore a clinical concern. Standard side effects of high-dose IFN therapy resemble symptoms of auto-immune diseases, which may make prompt diagnosis difficult. Therefore, it is important that auto-immune diseases such as dermatomyositis are recognized as potential side effects of treatment with high-dose IFNalpha.

Autoimmune Diseases↗
PubMed2001

Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature.

We describe a 42-year-old man with dermatomyositis and interstitial lung disease who presented with anterior neck pain and dyspnoea. Chest radiographs showed subcutaneous emphysema, pneumomediastinum and diffuse reticulonodular infiltration in both lungs. After the administration of high doses of prednisolone, an improvement of pulmonary function and respiratory symptoms was observed but the pneumomediastinum persists 12 months after diagnosis, and without any complication. We review the cases that have been reported thus far of pneumomediastinum associated with dermatomyositis and discuss the possible mechanisms involved. We conclude that pneumomediastinum is not an uncommon complication of dermatomyositis and that its aetiopathogenesis remains very unclear.

Adult↗
PubMed1994

[High dosage intravenous gamma globulin therapy in juvenile dermatomyositis].

In the last few years high-dose intravenous therapy with gammaglobulin in juvenile dermatomyositis with steroid resistant myopathy has been discussed as an alternative to cytostatic therapy. The 12-year-old Filipinogirl suffered from dermatomyositis with pronounced weakness of the limbs and masticatory muscles; creatinine-kinase was increased 22-fold. After 5 weeks of therapy with prednisolone (1.5 mg/kg body/weight/daily), the creatinine kinase decreased to sixfold of its standard value; the muscular weakness, however, progressed to almost complete paralysis of the scapular abductors and rotators and the coxofemoral flectors and rotators. Additional therapy with gammaglobulin at a dose of 1 g/kg body weight on two consecutive days at 4-week intervals led to complete restoration of muscular strength, normalization of muscle enzymes, and stabilization of these results after 12 courses of gammaglobulin and discontinuation of the steroid medication after 8 courses. This observation suggests that high-dose intravenous application of gammaglobulin represents an efficient therapy with minimal side effects in steroid-resistant juvenile dermatomyositis.

Biopsy↗
PubMed1995

[Dermatomyositis-like skin changes with long-term hydroxyurea (Litalir) therapy].

We report on dermatomyositis-like adverse cutaneous reactions following long-term maintenance therapy with hydroxyurea in two patients suffering from chronic myelogenous leukaemia (CML). In addition to non-specific side effects, such as xerosis, pruritus and hyperpigmentation, both patients presented with more specific skin changes, i.e. erythematous lesions, scaling, and partially atrophic areas distributed in a linear fashion on the dorsal aspects of the hands and fingers. In addition, teleangiectatic erythema of the face was present in both patients, and this was associated with oedema of the eyelids in one patient. Despite these dermatomyositis-like features there were no clinical signs of muscular involvement, and muscle-specific enzymes were within normal ranges. Skin biopsy specimens revealed an interface dermatitis characterized by a lichenoid cell infiltrate, vacuolar alteration of basal cells, necrotic keratinocytes within the spinous zone, focal hypergranulosis, ortho-hyperkeratosis and telangiectases in the upper part of the dermis. Analogous histopathological findings have been documented in lichen planus-like skin changes on the hands following hydroxyurea therapy. It seems doubtful whether there are actually any major differences between those skin changes described as dermatomyositis-like and those interpreted as lichen planus-like in patients receiving long-term hydroxyurea therapy.

Aged↗
PubMed2006

Contrast-enhanced ultrasound in dermatomyositis- and polymyositis.

OBJECTIVE: To evaluate prospectively contrast-enhanced ultrasound (CEUS) in patients suspected of having dermatomyositis or polymyositis. METHODS: In 35 patients (23 women, 12 men; mean age, 51 years+/-16 years) who were suspected of having dermatomyositis or polymyositis, perfusion in clinically affected skeletal muscles was quantified with contrast-enhanced intermittent power Doppler ultrasound. By applying a modified model that analyzed the replenishment kinetics of microbubbles, the perfusion-related parameters blood flow, local blood volume and blood flow velocity were measured. Findings were compared with muscle biopsy appearances and with the results of MRI that was performed with a 1.5-Tesla unit. Receiver operating characteristic analysis was performed and optimum thresholds for diagnosis of myositis were determined. RESULTS: Eleven patients had histologically confirmed dermatomyositis or polymyositis and showed significantly higher blood flow velocity (P=.01 for dermato- and P<.001 for polymyositis), blood flow (P<.001 for dermato- and polymyositis), and blood volume (P=.007 for dermato- and P<.001 for polymyositis) on contrast-enhanced ultrasound than those who did not have myositis. An increase in signal intensity on T2-weighted MR images was found in all patients with myositis. MRI had a sensitivity, specificity, positive (PPV), and negative predicting values (NPV) of 100%, 88%, 77%, and 100% for diagnosis of myositis, respectively. CEUS blood flow was the best ultrasound measure for diagnosis of dermato- or polymyositis with sensitivity, specificity, PPV, and NPV of 73%, 91%, 80%, and 88%, respectively. CONCLUSIONS: Increased skeletal muscle perfusion measured by CEUS could serve as an additional measurer for the diagnosis of an inflammatory myopathy.

Adult↗
PubMed2004

Dermatomyositis associated with lymphoproliferative disorder of NK cells and occult small cell lung carcinoma.

We report a case of dermatomyositis that was associated with a marked expansion of circulating NK cells (CD3-CD16+) with homogeneous phenotype (CD56- CD94- CD159a- CD244+) which satisfied the updated diagnostic criteria for the lymphoproliferative disorder of granular lymphocytes/NK cells. Immunosuppressive treatment induced remission of the dermatomyositis and decreased the number of circulating NK cells. However, a small cell lung carcinoma, that was occult at the time of the presentation of dermatomyositis, was diagnosed 10 months thereafter.

Aged↗
PubMed2006

Cytomegalovirus retinitis in a patient with dermatomyositis.

Cytomegalovirus (CMV) retinitis rarely occurs in patients with connective tissue disease. We present a rare case of CMV retinitis in a patient with dermatomyositis. A 38-year-old man was admitted due to blurred vision and floaters of right eye. He had been diagnosed with dermatomyositis and taken prednisolone and immunosuppressive agents for 10 months. He was diagnosed as having CMV retinitis based upon ophthalmologic examination, decreased CD4+T cell count, and positive IgM anti-CMV antibody. Intravenous ganciclovir was administrated with cessation of immunosuppressive agents for 40 days and the lesion did not progress. This is the first report of a CMV retinitis that developed in a patient with dermatomyositis.

Adult↗
PubMed2006

Successful treatment of refractory rash in paraneoplastic amyopathic dermatomyositis.

Skin involvement in dermatomyositis does not always parallel muscle disease and can be the most active or severe component of the disease, failing to respond to therapeutic interventions that are adequate for myositis and other systemic involvement. This case refers to a resistant paraneoplastic amyopathic dermatomyositis rash which readily resolved with intravenous immunoglobulin (IVIG), practically in the form of monotherapy. This observation gains particular significance considering that IVIG is still regarded as a second-line therapeutic regimen in the treatment of dermatomyositis despite its prevalence in matters of safety.

Adult↗
PubMed2001

Induction of remission with intravenous immunoglobulin and cyclophosphamide in steroid-resistant Evans' syndrome associated with dermatomyositis.

Evans' syndrome is characterised by the simultaneous or sequential occurrence of Coombs'-positive haemolytic anaemia (AIHA) and immune thrombocytopenia without underlying aetiology. It has been found to be associated with collagen vascular diseases, especially systemic lupus erythematosus (SLE) and scleroderma. However, Evans' syndrome with dermatomyositis is very rare. A 59-year-old woman, who had been taking high-dose prednisolone for a month and cyclosporin for 10 days for dermatomyositis, developed purpura on the left popliteal fossa. The platelet and haemoglobin levels decreased to 77,000/mm3 and 9.8 g/dl, respectively. Antiplatelet antibody was positive. Thrombocytopenia responded to intravenous immunoglobulin (IVIG) for a short time, but further decreased in a week. Her blood film showed features of haemolytic anaemia. Laboratory findings showed reticulocytosis and a positive direct Coombs' test. Bone marrow examination showed a mild hyperplasia of erythroid precursors and megakaryocytes. The patient was successfully treated with cyclophosphamide in addition to oral prednisolone. AIHA in connective tissue disease may develop gradually and show a benign clinical course in most patients. Therefore, we suggest that patients with dermatomyositis and anaemia should always be checked for haemolysis if there is no other explanation.

Anemia, Hemolytic, Autoimmune↗
PubMed2001

Amyopathic dermatomyositis.

Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.

Adult↗
PubMed1976

Skin capillary abnormalities as indicators of organ involvement in scleroderma (systemic sclerosis), Raynaud's syndrome and dermatomyositis.

Forty-four study patients with scleroderma (systemic sclerosis) (28 patients), Raynaud's syndrome (13 patients) or dermatomyositis (three patients) were observed for skin capillary abnormalities by widefield microscopy and compared with three control groups of 20 subjects each: (1) patients with other rheumatic disease, (2) hospitalized patients with nonrheumatic conditions, and (3) healthy volunteers. The distinctive microvascular pattern (dilated and distorted capillary loops alternating with avascular areas) previously reported in scleroderma and dermatomyositis was observed almost exclusively in the study patients. The severity of capillary abnormalities varied among the diagnostic subgroups, and a positive correlation was found between the degree and extent of abnormal microvascular patterns and multisystem involvement. On this basis, widefield nailfold capillary observations are proposed as a simple, inexpensive, reproducible technic for making an improved early diagnosis and predicting multisystem involvement in scleroderma, Raynaud's syndrome and dermatomyositis, presently a group of loosely associated and overlapping connective tissue disorders which often defy early and precise diagnosis.

Adult↗
PubMed1983

Serologic evidence for acute toxoplasmosis in polymyositis-dermatomyositis. Increased frequency of specific anti-toxoplasma IgM antibodies.

Several case reports have suggested an association between acquired toxoplasmosis and polymyositis-dermatomyositis. Because the presence of anti-Toxoplasma IgM antibodies suggests recent infection, 58 patients with polymyositis-dermatomyositis (from two medical centers) were examined for the presence of IgM antibodies using a specific indirect immunofluorescent technique. Serum samples were also examined for antibodies using the Sabin-Feldman dye test and complement fixation methods. Of 58 patients with polymyositis-dermatomyositis, 29 (50 percent) had positive Sabin-Feldman dye test results and 14 (24 percent) had positive IgM immunofluorescent findings. This is higher than the expected frequency. None of the patients with negative Sabin-Feldman dye test results had IgM immunofluorescent antibodies. Furthermore, IgM immunofluorescent antibodies were associated with the presence and titer of both Sabin-Feldman dye test and complement fixation antibodies. Evidence that the presence of antinuclear antibody and rheumatoid factor did not influence these results is presented. Patients with muscular dystrophy and systemic lupus erythematosus (with or without myositis) did not have an increased frequency of anti-Toxoplasma IgM immunofluorescent antibodies.

Acute Disease↗
PubMed1986

Dermatomyositis without creatine kinase elevation. A poor prognostic sign.

Serum muscle enzyme levels are usually elevated in patients with untreated polymyositis and dermatomyositis. Creatine kinase is the muscle enzyme most often used to diagnose inflammatory myopathies. Seven patients with dermatomyositis and normal creatine kinase levels are described. Five of the seven patients had either an associated malignancy or severe interstitial lung disease. The one-year survival of the six patients followed for that length of time was 33 percent. A lack of creatine kinase elevation in patients with dermatomyositis is a poor prognostic sign.

Adenocarcinoma↗
PubMed1989

Severe secondary amyloidosis in a dog with dermatomyositis.

A male collie aged 5 years 10 months, which developed dermatomyositis at 2 months of age, died from severe secondary amyloidosis. Amyloid deposition was most severe in renal glomeruli and produced renal failure. Amyloidosis has been reported in man with immune-mediated disorders including rheumatoid arthritis, systemic lupus erythematosus and dermatomyositis. It is possible that the inflammation in this case of familial canine dermatomyositis may have predisposed to the development of amyloidosis.

Amyloidosis↗
PubMed1987

Dermatomyositis presenting with Ludwig's angina.

An interesting case of a patient with dermatomyositis and a superimposed Ludwig's angina is presented. Dermatomyositis is a disease that is poorly understood and has many presenting characteristics similar to facial cellulitis and infection. In the present case the rapid progression of the clinical course necessitated prompt intervention. It was virtually impossible to differentiate between signs of dermatomyositis and an infectious process. No clear cause of the facial swelling was determined, but it was presumably from an ulcerative lesion in the floor of the mouth.

Bacterial Infections↗
PubMed1988

Complement levels in dogs with familial canine dermatomyositis.

CH50, C4, C2, and C3 levels were evaluated in 7 dogs affected with dermatomyositis and in 22 control dogs. Dogs with dermatomyositis did not have clinical evidence of active disease at the time of serum collection for complement assays. No absolute complement component deficiency was identified in dermatomyositis-affected dogs in this study; however, a statistical difference in C2 was identified between control dogs of non-collie breeds and control collies, suggesting there may be a breed difference in complement levels.

Animals↗