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Results for “DERMATOMYOSITIS”

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At least 433 records · Page 24Linked to original sources
PubMed1995

Dermatomyositis: remission induced with combined oral cyclosporine and high-dose intravenous immune globulin.

Dermatomyositis is an uncommon idiopathic chronic inflammatory disorder. Oral corticosteroids are the treatment of choice. A few patients become resistant to steroids. We describe two patients who, after failure to respond to oral and high-dose intravenous corticosteroids, received a combination of oral cyclosporine and high-dose intravenous immune globulin, with apparent remission. The first patient was a 6-year-old girl with recurrent disease and vasculitis, despite prednisone therapy and normal muscle enzyme levels. The response was remarkable within 3 weeks of therapy. The second patient was a 30-year-old woman with progressive disease and secondary respiratory failure despite oral prednisone and methotrexate therapy. The response to treatment was optimal within 4 weeks. She received maintenance low-dose cyclosporine and a tapering dose of prednisone. The combination of high-dose immune globulin and cyclosporine can be useful and safe in the management of steroid-resistant dermatomyositis.

Administration, Oral↗
PubMed1996

The immunopathologic and inflammatory differences between dermatomyositis, polymyositis and sporadic inclusion body myositis.

In polymyositis and sporadic inclusion body myositis, clonal expansion of CD8+ cells which are primed to recognize previously unknown muscle antigens occurs. Compared with sporadic inclusion body myositis, however, in which the T-cell response may not be antigen driven, there is in polymyositis an overexpression of certain T-cell receptor gene families among the autoinvasive T-cells. Although studies on the endomysial expression of cytokines and cell adhesion molecules have provided additional support for the concept of an ongoing immune process, the site of sensitization and the mechanism by which the autoimmune process is triggered remains to be established. In dermatomyositis, a multiorgan disease, evidence exists that the complement-mediated microvascular injury by the putative antibody may not be limited to the endomysial vessels but may also involve the blood vessels in the dermis. The antigenic target on the endothelial cell in dermatomyositis patients and the pathogenic role of the recently studied anti-Mi-2 antibody directed against a helicase are still to be determined.

Dermatomyositis↗
PubMed2006

Incidental granular parakeratosis associated with dermatomyositis.

A 43-year-old patient presented with a 4-month history of intermittently occurring symmetrical and slightly scaling erythematous plaques in his zygomatic regions. Because of polymyositis in the patient's history, the skin lesions were thought to be a manifestation of dermatomyositis. The skin biopsy revealed a sparse superficial perivascular infiltrate of lymphocytes, which was associated with telangiectases and both discrete vacuolar alteration and smudging of the dermal-epidermal junction. These findings were consonant with dermatomyositis. In addition to these features focal granular parakeratosis was present. The histopathologic presence of granular parakeratotic corneocytes above a zone of orthokeratosis and the absence of clinical features of granular parakeratosis indicate the granular parakeratosis is incidental.

Adult↗
PubMedDate not supplied

Juvenile dermatomyositis presenting with periorbital edema.

Juvenile dermatomyositis is a rare disease that affects the skin and muscles. It often presents with a classic heliotrope eyelid rash. We present a case of juvenile dermatomyositis presenting with significant bilateral periorbital edema, with its complete resolution after systemic antiinflammatory therapy.

Adolescent↗
PubMed1992

Fulminant dermatomyositis after removal of a cancer.

Dermatomyositis developed suddenly in a diabetic patient with CREST syndrome after the removal of a malignant tumor. Scrupulous physical examination excluded further systemic or cutaneous involvement. We raise certain still unsolved aspects regarding the association between dermatomyositis and neoplastic disorders.

Breast Neoplasms↗
PubMed1993

Calcinosis cutis in juvenile dermatomyositis responsive to aluminum hydroxide treatment.

Calcinosis cutis is a frequent complication of juvenile dermatomyositis; however, its treatment remains unsatisfactory. We report a case of calcinosis cutis in juvenile dermatomyositis which was successfully treated with oral aluminum hydroxide. Almost complete clearing of calcinosis was observed after 8 months of therapy. There were no adverse effects from therapy. Aluminum hydroxide is worth trying in treating calcinosis cutis.

Administration, Oral↗
PubMed1998

An autopsy case of dermatomyositis with rapidly progressive diffuse alveolar damage.

A 47-year-old woman visited a clinic with dyspnea which had continued for two months and was followed by general fatigue and fever. Antibiotics were not effective. Edematous erythema occurred on her face, elbows, knees and feet, and she entered our hospital. A skin biopsy revealed interface dermatitis with severe edema and mucinosis in dermis. Diffuse bilateral infiltration was observed in the chest X-ray, and laboratory findings showed increased LDH, GPT, GOT and CPK. No antinuclear factor was detected. Her respiratory condition rapidly worsened, and she died eight days after hospitalization in spite of corticosteroid pulse therapy. The autopsy revealed that the main cause of death was diffuse alveolar damage (DAD). Interstitial pneumonia related to dermatomyositis is not histologically uniform; the response to the therapy depends on its histological type. The patients with dermatomyositis who have poor prognosis are clinically characterized by acute onset with general symptoms and less pronounced muscle weakness; they generally show DAD in their lungs. We need to establish a simple method for distinguishing histological types of interstitial pneumonia and adequate therapy for each one.

Alanine Transaminase↗
PubMed1998

Dermatomyositis and nasopharyngeal carcinoma.

A nasopharyngeal carcinoma was diagnosed in a 37-year-old man with a two year history of dermatomyositis. The physical and laboratory examinations excluded further medical problems. To our knowledge, this is the first case of dermatomyositis associated with nasopharyngeal carcinoma in a white Israeli Jewish patient.

Adult↗
PubMed2002

Pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis.

A case of pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis (DM) is described. The patient was a 38-year-old woman with a history of dermatomyositis for 3 months. Yellowish, hard, papulo-plaque lesions, which looked like those of pseudoxanthoma elasticum, were noted on her left axilla. Calcium deposition was confirmed by X-ray, histopathological, and electron microscopic examinations. Histopathological and histochemical examinations showed acicular calcium deposition in the middle and deep dermis surrounded by mucin. Electron microscopic examination revealed that the calcium deposition was not on collagen fibers. These morphological features were distinct from those of PXE. We proposed the possibility that degenerated mucin or degenerated elastic fiber might result in subsequent calcium deposition in reticular calcinosis in adult DM. The calcification clinically disappeared without any specific treatment except for prednisolone and cyclophosphamide.

Adult↗
PubMed1991

Dermatomyositis responding to treatment of associated toxoplasmosis.

We report a 59-year-old woman with severe dermatomyositis in association with high serum toxoplasma antibody titres who was successfully treated with pyrimethamine and sulphadiazine. This case supports an aetiological role for toxoplasmosis in the pathogenesis of some cases of dermatomyositis.

Dermatomyositis↗
PubMed1993

Subcutaneous changes in dermatomyositis.

We report the case of a 42-year-old woman with concomitant panniculitis and dermatomyositis. Painful, indurated lesions on the buttocks, thighs, arms, abdomen and breasts were associated with proximal muscle weakness. Skin biopsy revealed lobular panniculitis, and vacuolar degeneration of epidermal basal cells. Direct immunofluorescence was negative. Serum muscle enzyme (creatinine-phosphokinase) levels were elevated, and electromyography demonstrated a myositic process. Muscle biopsy showed an inflammatory myositis. These results were consistent with dermatomyositis associated with panniculitis. Only five cases of this association have been reported previously. The relationship between these two conditions is discussed.

Adult↗
PubMed1995

Hydroxyurea-induced dermatomyositis-like eruption.

Hydroxyurea is frequently used to treat myeloproliferative syndromes. Cutaneous lesions resembling those seen in dermatomyositis have rarely been reported in the course of treatment with hydroxyurea. We report six additional patients with this unusual adverse effect. All of the patients had a very typical and similar cutaneous eruption, with scaly, linear erythema on the dorsa of the hands. Leg ulceration occurred in two cases. Muscle involvement was never observed. One patient had unexplained lung disease. In all the others the disorder pursued a benign course, even when hydroxyurea was not withdrawn. Dermatomyositis-like lesions seem to be a not infrequent and characteristic adverse reaction to hydroxyurea. Investigations are not required, and the course is usually benign.

Aged↗
PubMed1995

Dermatomyositis presenting in pregnancy.

We report the onset of dermatomyositis in a woman pregnant 38-weeks who subsequently delivered a healthy infant. The disease improved rapidly following delivery. The association of dermatomyositis with pregnancy is unusual, and fetal outcome may be adversely affected.

Adult↗
PubMed1990

Clinical effects of cyclosporin in dermatomyositis.

Auto-immune mechanisms, mostly cell mediated, have been described as the basic abnormality in polymyositis and dermatomyositis. We report here the case of a patient affected with dermatopolymyositis, resistant to therapy, who was treated with corticosteroids and cyclosporin. She presented with an infiltrative, erythematous rash on the neck, eyelids and cheeks and weakness of the proximal limb and cervical muscles. The dramatic and persistent response to cyclosporin obtained in this case represents a clear demonstration of the action of this drug in cases of dermatomyositis.

Azathioprine↗
PubMed1994

Dermatomyositis associated with malignant melanoma--case report and review of the literature.

Dermatomyositis (DM) is a connective tissue disorder characterized by cutaneous and muscle involvement. It is a well recognized paraneoplastic syndrome and has been linked with malignancy in 15-34% of adult patients. The course of DM in such patients usually correlates closely with the activity of the underlying malignancy. We report a patient who developed DM 4 years after excision of a malignant melanoma (MM) from the back and 1 year before the diagnosis of metastatic disease. A literature review revealed that the association of dermatomyositis with MM is rare and consistent with a dismal prognosis.

Dermatomyositis↗
PubMed1996

Autoimmune haemolytic anaemia associated with dermatomyositis.

We report the case of a female patient who at presentation fulfilled the diagnostic criteria of dermatomyositis and also had an autoimmune haemolytic anaemia. Although autoimmune haemolysis is a well recognized feature of a number of autoimmune disorders, it has not previously been described in association with dermatomyositis.

Adult↗
PubMed1996

Dermatomyositis and flagellate erythema.

Flagellate erythema, described by Moulin in 1970 as reminiscent of the lesions produced by flogging and by other authors as zebra-like lesions, was noted first in association with bleomycin treatment, but later occasionally reported in relationship with dermatomyositis, mostly from Japan. It is characterized by linear, erythematous, cutaneous streaks, pruriginous or painful, arising on an oedematous background and disappearing spontaneously over several weeks. So far, fewer than five cases have been reported in association with dermatomyositis, but we now present a further one.

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