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PubMedDate not supplied

Infrapatellar hypertrichosis: an unusual cutaneous manifestation of juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is an uncommon inflammatory myopathy with characteristic cutaneous manifestations. Certain clinical features such as cutaneous calcification, lipoatrophy, and generalized hypertrichosis can occur in JDM but are uncommon in the adult form of the disease. We report a young girl who presented with dramatic infrapatellar hypertrichosis along with more classic clinical signs of JDM, including heliotrope periorbital eruption, photosensitivity, and profound proximal muscle weakness. The clinical course was favorable, with improvement of cutaneous and muscular abnormalities after corticosteroid treatment.

Child, Preschool↗
PubMed2001

Dermatomyositis in association with transitional cell carcinoma of the bladder.

There is a well-recognized association between the inflammatory dermatomyopathy, dermatomyositis, and underlying visceral malignancy in adults. It is most commonly found in association with malignancies arising in the lung, breast and stomach. We report a case found in association with transitional cell carcinoma of the bladder, a site where there have only been a handful of previous reports.

Carcinoma, Transitional Cell↗
PubMed2003

Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment.

OBJECTIVES: To assess the prevalence, clinical characteristics, and treatment options of patients with interstitial lung disease (ILD) in polymyositis and dermatomyositis (PM/DM). PATIENTS AND METHODS: Sixty-three consecutive patients with PM/DM underwent standardized screening. Patients with ILD were monitored prospectively, and graded immunosuppression was administered according to the rate of clinical progression. RESULTS: ILD was diagnosed in 20 of 63 patients (32%). Generally, the clinical and serologic findings of the anti-Jo1 syndrome were present. Follow-up evaluation disclosed either a progressive or a nonprogressive course. The 10 patients with progressive ILD were distinguished from the nonprogressive group by extensive ground-glass opacities on high-resolution computed tomography (HRCT) and by bronchoalveolar lavage (BAL) neutrophilia. Intravenous pulse cyclophosphamide prevented further progression in all 10 patients and led to some functional improvement. In the 10 patients without rapidly progressive lung disease, immunosuppression of moderate intensity stabilized pulmonary findings during a median 35 months of follow-up. CONCLUSIONS: The prevalence of ILD in our patients with PM/DM was 32%; this emphasizes the need for pulmonary screening in all PM/DM patients. Progressive disease, featuring ground-glass opacities on HRCT and an inflammatory BAL cell profile, is amenable to intensive immunosuppression. Conversely, patients who do not have these HRCT and BAL features appear to have a low risk of pulmonary deterioration. RELEVANCE: Because the treatment for ILD seems to depend on the rate of clinical progression, future therapeutic trials of lung disease in PM/DM should stratify patients accordingly.

Adult↗
PubMed2001

Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study.

We conducted a national, retrospective population-based cohort study of 705 patients hospitalized with a first diagnosis of dermatomyositis (DM) or polymyositis (PM) during 1982-1996 based on linkage of hospital discharge, cancer registration, and death records in Scotland. Risks of cancer were assessed by calculating standardized incidence ratios (SIR). A first malignancy was diagnosed concurrently or subsequently in 50 patients with DM (SIR 7.7, 95% CI 5.7-10.1), and 40 patients with PM (2.1, 1.5-2.9). Significantly elevated risks were observed for lung, cervix uteri, and ovarian cancer in patients with DM, and for Hodgkin's disease in patients with PM. The excess risk of cancer was highest around the time of diagnosis, and for patients with DM remained high for at least 2 years. Risks were elevated for both sexes but only significantly so for females, and were highest in patients aged 45-74 years at the time of diagnosis for DM and 15-44 for PM.

Adolescent↗
PubMed1995

[Spontaneous hemothorax in dermatomyositis and long-term glucocorticoid treatment].

A 65-year-old woman with dermatomyositis for which she had been treated for ten years with prednisone (latterly 15 mg daily) suddenly experienced severe pain in the left thoracolumbar region. Cardiovascular, pulmonary and vertebral causes of the pain were excluded. But serological tests indicated inflammatory disease and the haemoglobin concentration was low (10.4 g/dl). Left pleural effusions were repeatedly aspirated and some haemorrhagic fluid obtained (haematocrit 0.31 in blood, 0.28 in the pleural effusion). Five days after admission her cardiovascular status became unstable and she developed respiratory failure (haemoglobin 7.6 g/dl). Chest radiograph showed increased pleural effusion. Subsequent thoracotomy revealed a left coagulothorax which was removed and flushed. During this procedure severe bleeding occurred from a covered perforation of the descending aorta, 1.5 x 1.5 cm in size. Although the aortic wall was thin, there was no aneurysm but arteriosclerotic changes and an external erosion near an abscessing mediastinitis, originating from a chronic purulent pleuritis and bronchopneumonia. The severe blood loss caused circulatory failure from which the patient could not be resuscitated.

Aged↗
PubMed1988

[Extraosseous calcification in juvenile dermatomyositis. The ineffectiveness of EHDP].

A now 20-year-old man was first diagnosed as having dermatomyositis when aged 12 years. At that time he had muscular weakness and heart failure followed a year later by heterotopic calcifications. For over seven years he was treated with corticoids, at times also with azathioprine or methotrexate. Nonetheless the calcifications progressed. Because of the extensive calcifications, predominantly of the extremities, he is severely disabled and has been confined to a wheel-chair. For one year he was treated with EHDP (1-hydroxyethylidenediphosphonate), up to 16 mg/dl per day. In addition he received prednisolone (10 mg/d) and azathioprine (100 mg/d). But radiologically there has been further slight progression of the calcifications, indicating that EHDP has been ineffective in this patient.

Adult↗
PubMed1998

Tumoral calcinosis and atypical juvenile dermatomyositis: case report.

Tumoral calcinosis is a rare, benign calcification of soft tissues in periarticular areas. It can manifest as an isolated disease or as part of an underlying disorder. We report a case of a 6-year-old girl who presented with tumoral calcinosis of her left knee in association with atypical juvenile dermatomyositis. Finally, we discuss operative and non-operative treatment strategies of tumoral calcinosis in children and adults.

Adult↗
PubMed2002

Panniculitis: a cutaneous manifestation of dermatomyositis.

Specific and nonspecific panniculitis sometimes occurs during the course of connective tissue diseases. However, the association of panniculitis and dermatomyositis (DM) has only rarely been described. Previous reports have suggested that panniculitis may be an inherent part of DM but, because the cases reported are few, it is difficult to know the significance of this unusual association. We describe 2 patients with DM in whom panniculitis developed during the course of the disease, and discuss its clinical and diagnostic significance.

Adult↗
PubMed2002

Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis.

BACKGROUND: Long-term consequences of juvenile dermatomyositis (JDM) include onset of calcinosis and subsequent functional impairment. Historic incidence of calcinosis has been reported between approximately 23% and 70%. Recent reports note improved outcome with high-dose steroids, yet the incidence of calcinosis has remained above 30%. OBJECTIVE: We attempted to determine whether rapid, aggressive disease management can prevent calcinosis and improve functional outcome. METHODS: Medical records of children with JDM managed at a pediatric medical center during a 10-year period were reviewed to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modality, and (3) functional outcome and presence of calcinosis. RESULTS: A total of 21 female and 14 male subjects diagnosed with JDM met inclusion criteria, with a mean age of diagnosis of 7.6 +/- 3.9 years. Mean time from onset of symptoms to treatment was 6.6 +/- 8.2 months. Pulse intravenous methylprednisone (30 mg/kg daily) or high-dose prednisone was used in 31 of 35 patients. Patients who failed to respond within 6 weeks were started on a regimen of methotrexate (23/35). At follow-up, 5 patients had mild calcinosis (14%). Onset of calcinosis was associated with a longer time to diagnosis and treatment (30.6 vs 6 months, P =.003), a longer duration of elevated muscle enzymes (34 vs 12.6 months, P =.03), and longer disease duration (42.8 vs 22.2 months, P =.05). CONCLUSION: Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long range sequelae of JDM, including calcinosis.

Calcinosis↗
PubMed2003

Dermatomyositis and malignancy in Tunisia: a multicenter national retrospective study of 20 cases.

OBJECTIVE AND METHODS: The aim of our study was to report the epidemiologic, clinical, and biologic profiles of dermatomyositis (DM) associated with malignancy in patients from Tunisia. From January 1982 to January 2000, we collected retrospectively 20 case reports of DM associated with cancer from the different university hospital centers of Tunisia. Initial workup included anamnesis, clinical examination, cancer staging and classification, serum muscle enzymes (creatine phosphokinase, lactate dehydrogenase, aldolase, and transaminases), electromyography, and muscular biopsy. We calculated the median survival and mean value of all the variables. Comparisons of statistical tests were done with the Kruskal-Wallis test. RESULTS: Among the 130 DM cases of our study, 20 were associated with cancer (15.38%). The mean age of our patients was 49.6 years and the sex ratio (female/male) was equal to 3. Cancers were mainly those of the breast (35%) and nasopharynx (25%). DM followed a paraneoplastic course in 90% of the cases. The profile of seric muscular enzymes showed a significant statistical difference (P =.05) between a group of patients with severe muscular weakness and a group with moderate muscle weakness only for creatine kinase. The median survival was 36.5 months after diagnosis of DM and 48.6 months after that of cancer. The 5-year actuarial survival was 38% as related to cancer and 16% as related to DM. Mortality was 45%, in 90% as a result of cancer. CONCLUSIONS: In our study, nasopharyngeal carcinoma represents the second cancer associated with DM, after breast neoplasm, demonstrating the frequency of these 2 cancers in our country. Despite our reduced number of study samples, our study also suggests a relationship between severe muscle weakness and high seric muscle enzymes.

Adolescent↗
PubMedDate not supplied

Juvenile dermatomyositis: an atypical presentation.

Juvenile dermatomyositis (JDM) is a rare disease of unknown etiology that results in inflammation of the connective tissue of striated muscle, subcutaneous tissue, nail beds, and skin. Although JDM is a rare disease, occurring in 3 per 1 million children, it is the most common idiopathic, inflammatory, myopathic disease of childhood. A case example is provided that describes an atypical presentation of JDM. Information about the disease, the usual and unusual clinical manifestations, the diagnostic processes, and therapeutic management plans for JDM are presented. Results of a comparison between this case and the literature are discussed, along with implications for nurse practitioners.

Adrenal Cortex Hormones↗
PubMed2003

Fatal interstitial pulmonary fibrosis in anti-Jo-1-negative amyopathic dermatomyositis.

Amyopathic dermatomyositis (DM) describes a subpopulation with the cutaneous eruption of DM, but without muscle involvement. Interstitial pulmonary fibrosis is a recognized complication of DM, often correlated with antisynthetase enzymes, such as anti-Jo-1. We describe a case of fatal IPF in a patient with anti-Jo-1 antibody-negative amyopathic DM.

Antibodies, Antinuclear↗
PubMed2004

Pathological calcification in juvenile dermatomyositis (JDM): microCT and synchrotron x-ray diffraction reveal hydroxyapatite with varied microstructures.

The objective of this study was to begin to relate the microstructure of calcinosis samples to clinical and laboratory characteristics of the juvenile dermatomyositis (JDM) patients. Laboratory x-ray microCT (micro-Computed Tomography) noninvasively mapped microstructure for the first time in JDM calcifications. Synchrotron x-ray diffraction (transmission geometry) identified the mineral phase and crystallite size in the deposits. Samples were obtained from four children who had active JDM longer than 80 months and who were typed for TNFalpha-308 allele polymorphisms. Uniform mineral (giving the appearance of an extruded solid) was observed in one patient, and irregular blocks of differing sizes filled the samples from two other patients. The sample from the fourth patient appeared to combine features of the other two types. These spatial distributions of mineral were quite different from those in a bone reference sample. The only mineral observed in the JDM samples was hydroxyapatite (HAP), and the diffraction peaks of the JDM samples were slightly narrower than those of a trabecular bone reference sample. Diffraction peak widths of the JDM specimens revealed crystallite sizes (approximately 220-240 A) that are comparable to values reported in the literature for bone. Three children were positive for TNFalpha-308 GA polymorphism. The data suggest several possible origins for blocky vs. uniform structure of the JDM calcifications, including differences in duration of untreated inflammation, in TNFalpha-308 polymorphism, and in mechanical constraint at the calcification site. Information from additional samples is required to determine the relative role of each of these factors. Taken together, non-invasive microCT and x-ray diffraction characterization on the same samples offer an informative window into the dystrophic mineralization process in JDM.

Adolescent↗
PubMed2000

The safety of a resistive home exercise program in patients with recent onset active polymyositis or dermatomyositis.

The objective was to investigate whether a 12-week resistive home exercise program in addition to conventional medical treatment could be safely performed regarding muscle inflammation, muscle function, and quality of life in patients with active polymyositis (PM) or dermatomyositis (DM). Eleven patients diagnosed with active PM or DM were included. Muscle biopsies and Magnetic Resonance Imaging (MRI) of the thighs were performed. Quality of life, function, and subjective global disease impact (SGDI) were assessed and creatine phosphokinase levels (CPK) were analysed. The patients exercised with the exercise program for 15 minutes and took a 15-minute walk five days a week for 12 weeks. After the exercise period there was no sign of increased muscle inflammation. The group showed significantly improved function and quality of life compared to the start of study. It seems that this exercise program safely can be employed in patients with active PM or DM, and we suggest that physical exercise should be included in the rehabilitation of these patients.

Adult↗
PubMed2000

Cyclosporine A versus methotrexate in the treatment of polymyositis and dermatomyositis.

OBJECTIVE: To determine the effectiveness and tolerance of treatment with cyclosporine A (CyA) or methotrexate (MTX) added to corticosteroids in patients with severe, active polymyositis (PM) and dermatomyositis (DM). PATIENTS AND METHODS: Thirty-six patients (20 with DM, 16 with PM) were enrolled into the study and randomized in MTX (n = 17) and CyA (n = 19) groups. Muscle endurance and functional test (MEFT), clinical assessment (CA), global patient's assessment (GPA), muscle MRI, serum CK, myoglobin, IL-1Ra, and autoantibody status were used to assess the response to therapy after 1, 3, and 6 months. RESULTS: Significant improvement in MEFT, CA, GPA, and muscle MRI was found in both groups. Patients treated with MTX showed insignificantly better response than patients with CyA. CK levels in the MTX group decreased significantly after 1, 3, and 6 months, whereas a significant reduction in the CyA group was first observed after 6 months. IL-1Ra serum levels significantly dropped in the CyA group after two weeks, whereas in the MTX group the significant decrease was first seen after 3 months of treatment. Good correlation was found between each of the clinical parameters (MEFT, CA, and GPA), none of them showed any correlation with CK or IL-1Ra levels. CONCLUSIONS: Administration of MTX or CyA added to corticosteroids was associated with clinical and laboratory improvement. Changes in CK and IL-1Ra levels were not associated with parameters of clinical disease severity measured in this study.

Adrenal Cortex Hormones↗
PubMed2003

Successful treatment of dermatomyositis-related rapidly progressive interstitial pneumonitis with sequential oral cyclophosphamide and azathioprine.

Interstitial lung disease (ILD) is a major complication of idiopathic inflammatory myopathies (IIM). We report the successful use of oral cyclophosphamide, followed by azathioprine maintenance, in the treatment of a patient with dermatomyositis-related rapidly deteriorating ILD, resistant to steroid. Prompt recognition and early aggressive immunosuppressive therapy may improve the outlook of this condition.

Administration, Oral↗