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Results for “DERMATOMYOSITIS”

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PubMed1977

Tubular cytoplasmic inclusions in a case of childhood dermatomyositis with migratory subcutaneous nodules.

A boy with dermatomyositis which began at the age of one year and five months showed multiple migratory subcutaneous nodules, which have seldom been described. Histological and electron microscopic studies of muscles and subcutaneous nodules demonstrated the following interesting findings. 1. Light microscopy. The migratory subcutaneous nodules consisted of non-suppurative panniculitis and ischaemic adipo-necrosis as a sequel to vascular lesions. This finding suggests that the nodules may have arisen from the subcutaneous adipose tissue which had been severely damaged by systemic angiopathy. 2. Electron microscopy. Examination of the vessels in muscle and subcutaneous nodules showed tubular cytoplasmic inclusions with a diameter of approximately 250 A in the endoplasmic reticulum of vascular endothelial cells. These observations provide strong support for the concept that the fundamental pathologic process in childhood dermatomyositis is of a vascular nature, and the primary lesion is in the walls of the intramuscular blood vessels.

Adipose Tissue↗

PubMed1991

Dermatomyositis as presenting symptom of Hodgkin's disease.

The association between dermatomyositis and malignancy is well documented. However, the association between dermatomyositis and Hodgkin's disease is very unusual, particularly in children, adolescents, and young adults. A MEDLINE search of the literature revealed only 13 previous cases.

Adolescent↗

PubMed1990

Milk of calcium fluid collection in dermatomyositis: ultrasound findings.

Extensive subcutaneous and intermuscular calcium laden fluid collections (milk of calcium) were found in two young girls with severe dermatomyositis. Sonographic examination clearly showed the nature and extent of the collections. Knowledge of this new feature of dermatomyositis should help avoid confusing these fluid collections with soft tissue infection and deep abscess in these often steroid dependent children.

Body Fluids↗

PubMed1990

Correlation between tests of muscle involvement and clinical muscle weakness in polymyositis and dermatomyositis.

A correlation study was performed on the degree of muscle weakness in 36 patients with dermatomyositis and 69 with polymyositis in relation to muscle biopsy findings, electromyography (EMG) abnormalities, and serum concentrations of creatine kinase (CK), aspartate aminotransferase (AST) and alanine aminotransferase (ALT) enzymes. Statistically significant correlations were found between muscle weakness and EMG results in patients with polymyositis, and between muscle weakness and serum CK and AST levels in dermatomyositis. As expected, correlations were found between the results of the three enzyme determinations in both groups of patients.

Alanine Transaminase↗

PubMed1985

An autopsy case with Good's syndrome and dermatomyositis.

A 31-year-old male patient complaining of repeated respiratory infections was revealed to be in an immunodeficient state in both humoral and cellular immunity and accompanied by dermatomyositis which was proved by both clinical and histological findings. Persistent diarrhea followed by colonic bleeding due to multiple ulcers and a severe liver dysfunction seemed to bring him to his death. The autopsy revealed the presence of thymoma of spindle cell type, depleted cells in the systemic lymphatic systems and inflammatory lesions in the muscle, liver, lung, kidney and gastrointestinal tract. This is the first report of Good's syndrome accompanied by dermatomyositis.

Adult↗

PubMed1978

Dermatomyositis, suxamethonium action and atypical plasmacholinesterase.

A 19-year-old girl suffering from active dermatomyositis was given suxamethonium 60 mg during anaesthesia for termination of pregnancy. A prolonged suxamethonium action occurred which was explained by the finding of homozygous atypical plasmacholinesterase in her blood. Although no fasciculations were seen immediately after injection of the drug, a period of fasciculations progressing from the extremities to the head and trunk occurred during recovery of muscle tone. No hyperpyrexia or elevation of serum creatine phosphokinase occurred. This was ascribed to the steroid therapy she received. Plasma from four other patients suffering from dermatomyositis was also investigated and one young woman, also pregnant, was found to be heterozygous for the atypical enzyme.

Adult↗

PubMed1996

Extensive soft-tissue involvement of dermatomyositis detected by whole-body scintigraphy with 99mTc-MDP and 201TL-chloride.

The authors present a case of extensive soft-tissue radioactivity visualized on both 99mTc-MDP and 201Tl-chloride scintigrams in a patient with dermatomyositis and colon cancer. Incidentally, diffuse and intense uptake of 99mTc-MDP was observed in the shoulder girdles, anterior chest wall, psoas muscles, both proximal thighs and right lower limb, corresponding to the sites of symptomatic muscles, even though skin lesions were limited and no calcification was detected on radiographs. Moreover, 201Tl-chloride was also intensely accumulated in nearly the same sites as the symptomatic muscles as shown on the 99mTc-MDP bone scintigrams. Whole-body scintigraphy with 99mTc-MDP and 201Tl-chloride is a useful tool to detect occult muscle lesions with dystrophic calcification and hyperemia in dermatomyositis.

Calcinosis↗

PubMed1997

[Generalized Mycobacterium avium-intracellulare infection due to immunosuppressive therapy of paraneoplastic dermatomyositis].

The ubiquitous Mycobacterium avium-intracellulare (MAI) is the most frequent cause of disseminated atypical mycobacteriosis in AIDS patients. MAI infections may develop in patients with other acquired immune defects, such as connective tissue disorders. In adults, the gastrointestinal and respiratory systems are most frequently affected. We report a patient with dermatomyositis receiving immunosuppressive therapy in whom only the skin and the skeletal system were affected by MAI. Because it presented with polymyositis-like symptoms, the infection was initially not identified and treated. The MAI was cultured from a periarticular joint effusion from the right upper arm and from venous blood, as well as identified histologically in lesional skin. Resistance to antibiotics developed most likely because the patient failed to take oral antibiotics regularly. Because of an acute exacerbation of the tumor-associated dermatomyositis, immunosuppressive therapy was initiated, while the tuberculostatic therapy was continued. Using these therapies both diseases markedly improved. In patients with connective tissue disorders receiving longterm immunosuppressive therapy, especially when changes in symptoms and signs are observed, opportunistic infections such as MAI should be considered and included in the differential diagnosis.

Adenocarcinoma↗

PubMed2004

[Extraosseous calcifications in dermatomyositis. A rare differential diagnosis of a benign heterotropic ossification].

Juvenile dermatomyositis is rare but still the most common idiopathic inflammatory myopathy of the childhood. Operative treatment may be indicated, whenever joint motion and patients mobility is limited through the manifestation of calcinosis cutis. Although Vitamin K dependent coagulation pathways have been described, the entire pathophysiological mechanism of its occurrence is currently not clarified. Standard therapy includes the systemic application of steroids and cytotoxins, other options involve aluminum-hydroxid or diltiazem. Only in serious functional obstructions operative resection may be indicated. The case of a 39-year-old female with a 24 year history of dermatomyositis involving most areas of the upper and lower extremities like a coat of mail is presented. Surgical resection of the calcification revealed a good functional recovery of the joint but because of a high recurrency rate the operation is only indicated in special cases.

Adult↗

PubMed1999

[Juvenile dermatomyositis--acute recidivism or sepsis?].

A 22-year-old male with juvenile dermatomyositis presented with fever up to 40 degrees C and acute pain in his right thigh accompanied by muscle weakness, a skin rash and a tender swelling. Serum aspartate aminotransferase (AST) and aldolase were mildly elevated. C-reactive protein (CRP) and fibrinogen were markedly increased. The differential white blood cell count revealed relative lymphopenia. Radiography showed diffuse calcifications particularly around the thighs and knees of both legs. Magnetic resonance imaging (MRI) demonstrated inflammatory infiltrates in the right thigh. The lesions were identified as phlegmone by immunoszintigraphy with 99mTc-labelled antigranulocyte antibodies. On the 10th day of treatment Staphylococcus aureus was cultured from blood. Patients with juvenile dermatomyositis and calcinosis may develop bacterial infections of soft tissue which sometimes mimic a disease flare. For differential diagnosis plain radiographs, CT scans and MRI are of limited value. Immunoszintigraphy is able to differentiate between infiltrates caused by granulocytes and lymphocytes.

Adult↗

PubMed2006

Juvenile dermatomyositis with a rare and remarkable complication: sinus bradycardia.

Juvenile dermatomyositis (JDM) is characterized by proximal muscle weakness, vasculopathy of the skin and muscles, and typical skin rash. Approximately, 70% of adult patients with dermatomyositis have evidence of cardiac damage. However, cardiac involvement, one of the important causes of death, has been reported rarely in children with JDM. Here, we describe an 11-year-old boy who presented with bradycardia as a result of sinus node involvement due to JDM and recovered by the treatment of JDM without any specific drug for bradycardia.

Antirheumatic Agents↗

PubMed2006

Therapeutic options in autoimmune inflammatory myopathies (dermatomyositis, polymyositis, inclusion body myositis).

Polymyositis, dermatomyositis, and inclusion body myositis are idiopathic inflammatory myopathies of unknown etiology with autoimmune pathogenesis. For choosing an individual and efficient therapy, diagnostic assignment is an important factor. Therapeutic options in dermatomyositis and polymyositis include corticosteroids and immunosuppressives. Intravenous immunoglobulins are only needed in special cases. In inclusion body myositis, corticosteroids and immunosuppressives are not successful. At the moment intravenous immunoglobulins are the only therapeutic possibility.

Adrenal Cortex Hormones↗

PubMed2004

A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis.

Amyopathic dermatomyositis (ADM) is characterized by the typical cutaneous features of dermatomyositis and minor involvement of the skeletal muscles. A 50-year-old woman had fever, reddening and pain in the distal part of all four limbs, and cutaneous findings such as Gottron's papules and periorbital heliotrope. She showed no muscle weakness or atrophy, and her serum creatine kinase was within the normal range. Electromyography showed no myopathic pattern. Magnetic resonance imaging (MRI) recorded abnormal hyperintensity in the fascia and muscle of the tibialis anterior. A biopsy from the tibialis anterior muscle showed fasciitis and mild myopathic changes with focal perivascular infiltration. This patient also presented with interstitial pneumonitis, although evaluation for malignancy was negative. With steroid therapy, her symptoms and MRI abnormality disappeared within 2 months. This case is therefore considered to be a variant of ADM, presenting as dermato-fasciitis.

Anti-Inflammatory Agents↗

PubMed2006

Acute dermatomyositis with subcutaneous generalized edema.

The authors report a 40-year-old Caucasian man with relapsing muscle and skin involvement of dermatomyositis treated with high-dose corticosteroids, taken orally, and methotrexate and human gamma globulin, both administered intravenously. After 4 months of aggressive treatment, he presented with generalized edema, considered secondary to dermatomyositis. Aggressive immunosuppression did not stop disease progression. The literature concerning anasarca due to inflammatory myopathies is revised.

Acute Disease↗

PubMed2005

Prostate cancer underlying acute, definitive dermatomyositis: successful treatment with radical perineal prostatectomy.

The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation leading to progressive weakness of the proximal muscles. In 7-66% of cases of adult dermatomyositis different malignant tumours can promote the difficult cascade mechanisms at the cell level, leading to rapid weakness of skeletal muscles [1]. We report on a patient with all characteristic signs of acute, severe dermatomyositis associated with a low-grade, low-stage prostate cancer cured by radical perineal prostatectomy.

Adenocarcinoma↗

PubMed2000

Amyopathic dermatomyositis and pulmonary fibrosis.

'Amyotrophic dermatomyositis' (ADM) is used to describe a small subgroup of patients with the typical skin rash associated with dermatomyositis but without muscle involvement. Lung involvement in ADM is rare. We report on the management of a patient with ADM associated with pulmonary fibrosis at presentation, and her response to corticosteroid treatment.

Dermatomyositis↗

PubMed2005

Hyperdense pericardial effusion in dermatomyositis and contrast induced nephropathy.

Pericardial involvement in dermatomyositis is rare and usually asymptomatic. In many instances, a diagnosis of pericardial involvement is not found until autopsy. Renal failure associated with connective tissue disorders can result in or potentially exaggerate pericardial inflammation. We report an unusual case of high-density pericardial effusion in a patient with dermatomyositis consequent upon contrast nephropathy as demonstrated by computed tomography. High-density pericardial effusion can be a result of an insidious cause such as pericardial inflammation rather than the more usual causes such as coronary or cardiac perforation.

Acute Kidney Injury↗

PubMed2004

Mitochondrial abnormalities in dermatomyositis: characteristic pattern of neuropathology.

The objective of the work described in this paper was to evaluate mitochondrial abnormalities in perifascicular atrophic fibers in muscle biopsies from patients with dermatomyositis (DM). We localized cytochrome c oxidase (COX) and succinate dehydrogenase (SDH) histochemically in muscle biopsies of 12 patients with DM, and 12 control patients with neurogenic atrophy. These two histochemical techniques were also combined on single tissue sections in order to accentuate any COX-negative fibers. Eleven out of 12 patients (91.6%) with DM showed histochemical evidence of mitochondrial dysfunction in perifascicular distribution. Similar abnormalities in histochemical staining were not seen in comparably sized myofibers that were atrophic due to denervation. It is concluded that abnormal SDH and COX histochemical activities in atrophic perifascicular fibers are characteristic of dermatomyositis. These abnormal staining characteristics could not be accounted for solely by myofiber atrophy, or by generalized abnormalities in histochemical staining.

Adolescent↗