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PubMed1986

Dermatomyositis-polymyositis in children.

Sixteen cases of dermatomyositis-polymyositis in children (dermatomyositis 15; polymyositis 1) have been reviewed. Proximal muscle weakness with a characteristic skin rash was vital for diagnosis in the majority of children. The positive laboratory tests such as creatine phosphokinase (CPK), electromyography (EMG) and muscle biopsy were helpful but results were normal in some cases even in the acute phase of the illness. All but one had been treated initially with high dose corticosteroids followed by a low dose maintenance for a prolonged period. Two in addition had had cytotoxic agents. Thirteen of the sixteen were currently in remission. In the other three cases, one with polymyositis only, the disease remained active and the two with dermatomyositis died. Only three of the patients were incapacitated by residual fixed contractures and extruding calcinosis. On the whole the prognosis of dermatomyositis is good.

Adolescent↗
PubMed1993

Fatal pneumomediastinum in dermatomyositis without creatine kinase elevation.

A 41-year-old woman developed fatal pneumomediastinum in the course of dermatomyositis. One characteristic feature of this patient was the relatively mild myositis with slight elevation of serum creatine kinase. In a literature review, some of the patients with dermatomyositis, but not polymyositis, with a very slight elevation of serum creatine kinase tended to develop spontaneous pneumomediastinum during corticosteroid therapy. Since pneumomediastinum is a highly fatal complication in patients with dermatomyositis, only slight elevation of creatine kinase could be a marker for poor prognosis. Thus, mild/minimal myositis in patients with dermatomyositis should be carefully observed irrespective of corticosteroid therapy.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Psoriasis in a patient with dermatomyositis.

Psoriasis has been consistently associated with arthritis and inflammatory bowel diseases, though there have been reports on patients with psoriasis and other autoimmune dermatoses. Sometimes, sharply demarcated scaly plaques located over extensor surfaces in patients with dermatomyositis may clinically resemble psoriatic lesions. Histologic findings of interface dermatitis, typical for dermatomyositis, help clinicians to rule out psoriasis. A patient is presented with dermatomyositis in which psoriatic lesions developed over the extremities and lower trunk. Histological examination confirmed the diagnosis of psoriasis. Both diseases have run independent courses. It is prudent to include psoriasis in the differential diagnosis of scaly eruptions occurring in dermatomyositis patients.

Dermatomyositis↗
PubMed2006

Dermatomyositis associated with hepatocellular carcinoma in an elderly female patient with hepatitis C virus-related liver cirrhosis.

A 79-year-old female patient with hepatitis C virus-related liver cirrhosis was diagnosed as having hepatocellular carcinoma (HCC) with a diameter of 2.0 cm. She refused therapy for HCC. Nine months after the diagnosis, she developed dermatomyositis when the HCC enlarged to a diameter of 6.0 cm. She underwent therapy for dermatomyositis, and then transcatheter arterial chemoembolization for HCC. Although the manifestations of dermatomyositis improved and entire tumor necrosis was achieved, she died of pneumonia 2 mo after the treatment of HCC. HCC and/or chronic hepatitis C virus infection might be involved in the pathogenesis of dermatomyositis.

Aged↗
PubMedDate not supplied

High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels.

An open study was conducted to identify and investigate dermatomyositis patients who benefit from IVIG treatment, based on dermatological criteria, myositis-related symptoms and immune/inflammatory parameters. 19 patients (16 females and three males, ages 31-84) suffered from dermatomyositis, and 4/19 patients had paraneoplastic dermatomyositis. We monitored the disease activity by documenting the clinical symptoms, recording muscle-related parameters (electromyography, serum creatine kinase, histopathology), and by determining circulating autoantibodies and serum levels of IL-6, sIL-2R, sTNF-a-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate muscle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders had severe skin and muscle disease, concomitant with autoantibodies and/or malignancy. sIL-2R levels were initially elevated in all patients but reverted to normal in IVIG-responders only. Creatine kinase-levels and other parameters did not correlate with disease activity and/or treatment response. IVIG is effective in selected dermatomyositis patients. sIL-2R serum levels appear to be useful predictors of IVIG-induced treatment response and disease activity.

Adult↗
PubMed2000

[Intravenous immunoglobulins in the treatment of polymyositis and dermatomyositis].

Polymyositis and dermatomyositis are two inflammatory muscle diseases of unknown origin mediated by a dysimmune mechanism via a cytotoxic effect against the muscle fiber in polymyositis and a humoral effect against the muscle vessels in dermatomyositis. First line treatment of polymyositis and dermatomyositis is still based on corticosteroid therapy. In case of failure, corticosteroid dependence, or intolerance, a second line treatment using immunosuppressors or polyvalent human intravenous immunoglobulins (IVIg) is usually associated. We report our experience with IVIg in the treatment of 50 patients with myositis unresponsive to corticosteroids and immunosuppressors. Thirty-five patients had polymyositis and 15 had dermatomyositis. We discuss the possible mechanisms and their precise role in the therapeutic armamentarium in these dysimmune diseases.

Adrenal Cortex Hormones↗
PubMed2000

Sarcoidosis and dermatomyositis in a patient with hemoglobin SC. A case report and literature review.

Although sarcoidosis has occasionally been associated with hemoglobinopathies, its association with dermatomyositis is extremely rare. A 24 y/o African American male with hemoglobin SC disease developed proximal weakness. He had heliotrope rash, muscle weakness, elevated serum CK, myositis by EMG, and no malignancy. A muscle biopsy confirmed dermatomyositis. Two years later, he was hospitalized with constitutional and respiratory symptoms and parotid enlargement. Chest X-ray and CT scan showed diffuse micronodular infiltration in both lungs and mediastinal lymphadenopathy. A transbronchial lung biopsy revealed non-caseating granulomata consistent with sarcoidosis. AFB and fungal stains and cultures were negative. Thus, in this case, sarcoidosis developed after or in association with dermatomyositis. A review of world's literature showed five other non-identical cases of dermatomyositis associated with sarcoidosis.

Adult↗
PubMed2001

[Gastric cancer associated with dermatomyositis accompanied by photoallergy].

A 62-year-old man was admitted to the hospital because of a 5-month history of edematous erythema and itching on the face, upper chest, and upper extremities. The symptoms developed immediately after bathing in the sea. Dermatomyositis associated with photoallergy was diagnosed by skin and muscle biopsy. A search for malignancy revealed Borrmann 3 gastric cancer, and subtotal gastrectomy was performed. He has done well for 3 years and 8 months after the operation, but there has been no remission in the symptoms of dermatomyositis. Dermatomyositis associated with photoallergy has a higher incidence of complications with malignant disease than ordinary dermatomyositis.

Dermatitis, Photoallergic↗
PubMedDate not supplied

Topical FK506 (tacrolimus) therapy for facial erythematous lesions of cutaneous lupus erythematosus and dermatomyositis.

Tacrolimus is a prototype of a class of topical immunosuppressive agents with great potential for the treatment of inflammatory skin diseases. Topical tacrolimus therapy was applied to facial skin lesions in 11 cases of cutaneous lupus erythematosus (LE) and dermatomyositis. Of the 11 patients, 6 (3 systemic LE, one discoid LE and 2 dermatomyositis) showed a marked regression of their skin lesions after tacrolimus therapy, but 4 patients (3 discoid LE and one dermatomyositis) were resistant to the therapy. A good response was observed for facial erythematous lesions with edematous or telangiectatic changes in systemic LE and dermatomyositis. In discoid LE with typical discoid lesions, tacrolimus brought no improvement. Topical tacrolimus will become a new tool for managing the skin lesions of collagen diseases.

Administration, Topical↗
PubMed2001

Juvenile dermatomyositis in Thai children.

Juvenile dermatomyositis is a rare, chronic multisystemic inflammatory disorder of unknown etiology, characterized by a typical skin rash and proximal muscle weakness. A retrospective study from the medical records of patients diagnosed as juvenile dermatomyositis was performed at Queen Sirikit National Institute of Child Health from 1988 to 1998. There were seven cases of juvenile dermatomyositis diagnosed according to the criteria of Bohan and Peter. Six cases were female and one case was male. The age of diagnosis ranged from 2.5 years to 11 years. (mean age was 7 +/- 3.6 years). The presenting symptoms were muscle weakness (6 cases), muscle pain (2 cases) and skin rashes (4 cases). All of the patients developed proximal muscle weakness of the lower extremities varying from grade 3 to grade 4. The cutaneous manifestations were heliotrope signs (6 cases), gottron's papules (2 cases), photosensitivity (2 cases) and calcinosis cutis (4 cases). Electromyography (EMG) was performed in 6 cases and revealed typical change of myopathic type. Elevated muscle enzymes were noted in all cases. Muscle biopsy was performed in 6 cases and was compatible with myositis. Oral prednisolone (1-2 mg/kg/day) was given in 6 cases and the muscle weakness improved. There was no mortality in this study. Four cases developed calcinosis cutis 1 to 3 years after muscle weakness and did not respond to any treatment. In conclusion, juvenile dermatomyositis is a disease which causes chronic disability in children. Early diagnosis and treatment can prevent morbidity and mortality. Calcification at the skin usually occurs after the onset of muscle weakness several months to years after diagnosis.

Child↗
PubMed2002

[Amyopathic dermatomyositis].

Dermatomyositis is a systemic autoimmune disease which belongs to the group of idiopathic inflammatory myopathies. The disease is rare with an incidence of 0.1-1/100,000 and a prevalence of 1-6/100,000. Women are affected twice as often as men. In some patients the disease presents with dermatologic changes weeks to months before the myopathy arises. It was observed that in some patients the myositis develops much later and sometimes not at all. Therefore, the term of dermatomyositis sine myositis used in earlier literature has been changed to amyopathic dermatomyositis. The most important question is whether the patient needs systemic therapy with its possible side effects yet possibly preventing the appearance of myositis or only local therapy for the skin manifestations is necessary. The goal of this article is to summarize the latest findings in amyopathic dermatomyositis.

Dermatomyositis↗
PubMed2001

Juvenile dermatomyositis: literature review and report of a case.

Dermatomyositis is a rare disease of unknown origin, which affects both children and adults. In the juvenile form, dermatomyositis is a multisystem disease, characterized by myositis; an erythematous rash over the bridge of the nose, around the eyes, and on the trunk and limbs; vasculitis; and dilatation of the capillaries in the nail beds and gum margin. Late development of calcinosis is seen in approximately two-thirds of the patients. This case describes a 5-year-old dental patient diagnosed with dermatomyositis. This article includes the clinical manifestations of dermatomyositis in the body and in the oral cavity as well as the considerations that should be taken when treating these patients.

Child, Preschool↗
PubMed2002

[Juvenile dermatomyositis in the Nice area: a retrospective study 1991-2001].

INTRODUCTION: Juvenile dermatomyositis is a rare disease. We conducted a retrospective chart review on patients with juvenile dermatomyositis diagnosed in the Nice area from 1991 to 2001. Our purpose was to review diagnosis criteria and treatment strategies. PATIENTS AND METHODS: The cases of juvenile dermatomyositis were identified by phone investigation of physicians of the departments of paediatrics, dermatology, rheumatology and internal medicine. RESULTS: Seven cases of juvenile dermatomyositis (sex ratio M/W: 0.75, medium age: 7.7 years) were identified. Myalgia and/or weakness were the main reasons for initial consultation. Cutaneous lesions were present in all patients on initial presentation. Muscular enzymes were abnormal in 4 cases out of 7. Muscular biopsy was conducted in 6 patients. In one case, MRI revealed an inflammatory involvement although no clinical or biological sign existed. Systemic corticosteroids was the initial treatment, associated with monthly intravenous immunoglobulins in 5 cases, allowing initial control of the disease in all cases. The other therapies were: methotrexate (3 cases), ciclosporin A (1 case), and chloroquine (2 cases). The evolution was monocyclic in three cases, polycyclic in four cases. With a median follow-up of two years, all the patients are alive: five under treatment, three still in first flare. COMMENT: The diagnosis strategy seems relevant because at least 3 criteria of Peter and Bohan were found in 6/7 patients. Muscular biopsy appears fundamental in the diagnosis strategy. MRI was useful when it was conducted but its use has to be assessed. Therapies were those of previous published studies, except for the use of intravenous immunoglobulins as first-line treatment, associated with corticosteroids. This strategy may be justified for corticosteroid-sparing purposes. CONCLUSION: Working on a real, well-codified strategy of diagnosis and treatment would enhance the uniform management of these patients.

Adolescent↗
PubMed2003

[Cutaneous necrosis is predictive of cancer in adult dermatomyositis].

INTRODUCTION: Adult dermatomyositis is associated with cancer in 15 p. 100 to 50 p. 100 of cases and, hence, investigations should be systematically performed to search for cancer. A number of predictive factors have been reported. The aim of our study was to search for predictive factors of cancer, among adults with dermatomyositis. METHODS: We prospectively assessed 26 adults presenting with dermatomyositis, hospitalised in our department of dermatology from January 1993 to June 2000. The parameters assessed were: association with a cancer, age, gender, cutaneous necrosis, muscular weakness, electromyographic abnormalities, erythrocyte sedimentation rate, and muscular enzyme levels. RESULTS: Mean age was of 52 years and sex ratio (M/F) was of 0.53. Cancers were diagnosed in eight cases (31 p. 100) (mean age: 59.5 years; sex ratio=1; cancer localization: lung (2), breast (2), ovary, endometrium, bladder, and melanoma). Five patients in the cancer group had cutaneous necrosis and only 2 in the without cancer (p=0.01; PPV=71.4 p.100). Elevation of muscular enzyme was also associated with cancer. CONCLUSION: Our report demonstrates that cutaneous necrosis is closely associated with cancer and it suggests that in selected patients with dermatomyositis and cutaneous necrosis, more exhaustive and repeated investigations should be performed to search for cancer. The interest of elevation in muscular enzyme as a predictive factor of cancer is discussed.

Adult↗
PubMedDate not supplied

[Dermatomyositis and pregnancy].

INTRODUCTION: Dermatomyositis is a disorder known as a pathology whose etiology remains unknown. It can occur at any age with clinical symptoms of weakness and wasting, especially of the proximal musculature, due to inflammatory infiltration of muscles and destruction of muscle fibers. It is exceptionally seen during pregnancy, conditioning adverse effects in pregnant women. OBJECTIVE: The principal objective here is the presentation of a clinical case of dermatomyositis during pregnancy. CASE REPORT: Women of 33 years of age with term pregnancy, suffering from dermatomyositis from infancy, with satisfactory evolution. CONCLUSION: Dermitomyositis is a pathology of unknown origin whose incidence averages five cases in one million habitants with exceptional presentation during pregnancy. There are 29 case reports in the world literature. Perinatal morbimortality lies between 46 and 57% in case of activity. Fetal morbidity consists of intrauterine growth restriction prematurely and fetal death. Dermatomyositis has been associated with neoplasm LES and collagen disorders.

Adult↗
PubMed1991

Clinical, serologic, and immunogenetic studies in patients with dermatomyositis.

Dermatomyositis is a disease of unknown etiology characterized by progressive, symmetrical, proximal muscle weakness with accompanying compatible cutaneous findings. Thirty-nine patients with dermatomyositis from the Louisville, Kentucky area were enrolled in this study. Patients were grouped into those with or without a malignancy. Ten patients (26%) either had or have had a malignancy. Twenty-five Caucasian patients were HLA typed for the A, B, DR and DQ locus antigens, of whom 5 had an associated malignancy and 20 did not have a malignancy. We found that no single antigen had a significantly increased or decreased frequency as compared with our control population for the entire group, or for any clinical subset we examined. Serologic testing revealed 4 patients with anti-Mi-2 antibodies and 1 patient with anti-PM-SCL antibodies. No patient had a positive anti-Jo-1 antibody in this group. The results of serologic tests in this group did not correlate with any clinical subset or HLA antigen. Our findings were in agreement with the previous reports in which approximately 25% of patients with DM have an associated malignancy. Our findings also support the notion that untargeted malignancy searches are not warranted. Contrary to previous reports we did not observe an inverse relationship between cancer and pulmonary disease in the dermatomyositis patient. This study does not indicate that there are any HLA associations or clinical associations, other than age, that distinguish patients with dermatomyositis as running a greater risk of developing malignancy.

Dermatomyositis↗
PubMed2006

Dermatomyositis: immunopathologic study of skin lesions.

BACKGROUND: The pathogenesis of the inflammatory processes in the skin of dermatomyositis patients remains unclear. The aim of this study was to investigate the patterns of proliferation and apoptosis of epidermal keratinocytes and dermal infiltrating cells in DM patients. MATERIAL AND METHODS: Seventeen skin biopsy specimens from patients with dermatomyositis, which fulfilled the diagnostic criteria of Bohan and Peter, and Euwer and Sontheimer, were immunohistochemically investigated with monoclonal antibodies against human Ki-67, Bcl-2, CD3, CD4, anti-CD 45RO and a reaction was detected by streptovidine-biotin complex (Uni-Pak ABC). RESULTS: The lesional skin showed a marked increase of Ki-67-positive keratinocytes, which were predominantly located in the basal and germinative layer, but a lack of cells in areas of vacuolar degeneration and epidermal atrophy. A drastic reduction in the number of Bcl-2-positive cells localized in the basal cell compartment was observed. Antigen activated T lymphocytes (CD 45RO+) and CD3+ cells were in higher prevalence in dermal perivascular infiltrates of the affected skin. CONCLUSION: The defective regulation of apoptosis may play an important role in the development of cutaneous lesions of patients with dermatomyositis, in which the skin is a prominent target organ. Abnormal expression of Ki-67 and diminution of Bcl-2 in the epidermis, coupled with the perivascular location of T cells in the dermis are crucial keys to the histological diagnosis of dermatomyositis.

Adult↗
PubMed1989

[Dermatomyositis associated with normal serum muscle enzyme levels].

Cases of polymyositis or dermatomyositis in which levels of all the serum muscle enzymes are within the normal range throughout the entire clinical course are very rare. It has recently been suggested that when there is no increase in creatine kinase the prognosis in dermatomyositis is poor and there is a higher incidence of malignancy and interstitial lung disease associated with the condition. We describe a rare case of dermatomyositis without increase in serum muscle enzymes, including creatine kinase. No evidence of malignancy or interstitial lung disease was found after a followup of 18 months. In accordance with the report of a similar case, we suggest that the absence of an increase in creatine kinase in dermatomyositis is not necessarily a poor prognostic sign.

Adult↗