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PubMedDate not supplied

[Regression of primary melanoma with metastases associated with amyopathic dermatomyositis].

BACKGROUND: Dermatomyositis is a rare and serious inflammatory connective tissue disease characterized by a typical cutaneous rash and myopathy. Amyopathic dermatomyositis is a particular form of dermatomyositis involving only cutaneous signs and without myopathy present for over 2 years. PATIENTS AND METHODS: A 48 year-old woman presented with a 3-year history of cutaneous rash without myopathy characteristic of amyopathic dermatomyositis. Clinical examination revealed extensive axillary adenopathy, histological examination of which suggested secondary melanoma. The patient reported a black nevus in the axillary area that had disappeared 1 year earlier. Curettage of the lymph node was negative and the patient was treated with interferon (3M 3 times a week). Regression of the cutaneous signs was noted. DISCUSSION: The data, there have been no other reports of paraneoplastic amyopathic dermatomyositis associated with regression of primary melanoma. The literature contains few reports of dermatomyositis associated with melanoma. Amyopathic dermatomyositis may be associated with malignancy.

Axilla↗
PubMed1992

Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study.

BACKGROUND: An association between polymyositis and cancer was first proposed in 1916, but the existence of the association has been disputed. An association between dermatomyositis and cancer is better accepted, but its magnitude is not known. METHODS: We undertook a study to provide accurate estimates of the risk of cancer in patients with dermatomyositis or polymyositis. We studied the incidence of cancer and the rate of mortality from cancer in a population-based cohort of 788 patients with dermatomyositis or polymyositis in Sweden from 1963 through 1983. The results were compared with those for the general population. RESULTS: Among the 396 patients with polymyositis, 42 cancers were diagnosed at the same time or after polymyositis was diagnosed in 37 patients (9 percent). The relative risk of cancer was 1.8 (95 percent confidence interval, 1.1 to 2.7) in the male patients and 1.7 (95 percent confidence interval, 1.0 to 2.5) in the female patients. Eighty-four males and 85 females died, and in 24 of these cases (14 percent) cancer was the principal cause of death. The mortality ratio (the rate of mortality from cancer in these patients as compared with that in the general population) was 0.90 (95 percent confidence interval, 0.6 to 1.4). Among the 392 patients with dermatomyositis, 61 cancers were diagnosed at the same time or after dermatomyositis was diagnosed in 59 patients (15 percent). The relative risk of cancer was 2.4 (95 percent confidence interval, 1.6 to 3.6) in the male patients and 3.4 (95 percent confidence interval, 2.4 to 4.7) in the female patients. Fifty-seven males and 110 females died, and in 67 of these cases (40 percent) cancer was the principal cause of death (mortality ratio, 3.8; 95 percent confidence interval, 2.9 to 4.8). CONCLUSIONS: The risk of cancer is increased in patients with polymyositis or dermatomyositis. In patients with dermatomyositis there is also a higher rate of mortality from cancer.

Confidence Intervals↗
PubMed2001

Severe perturbations of the blood T cell repertoire in polymyositis, but not dermatomyositis patients.

Polymyositis and dermatomyositis are diseases characterized by muscle weakness and muscle inflammatory infiltrates. Their pathogenesis remains unclear. A central role for endomysial autoaggressive CD8(+) T cells is suspected in polymyositis and for perivascular B cells in dermatomyositis. We compared the T cell repertoire of 10 polymyositis and 10 dermatomyositis patients by immunoscope, a method providing a global assessment of the T cell repertoire and a sensitive detection of clonal T cell expansions. Samples were analyzed qualitatively and quantitatively in the blood (unsorted cells and CD4(+) and CD8(+) cells) and in muscle infiltrates. Dramatic perturbations of the T cell repertoire were observed in the blood of polymyositis but not dermatomyositis patients (p < 0.0005), the latter being undistinguishable from controls. These perturbations were due to oligoclonal expansions of CD8(+) T cells and most blood clonal expansions were also found in muscle. These results indicate that the pathogenesis of polymyositis and dermatomyositis is different and reinforce the view that polymyositis but not dermatomyositis is an autoimmune CD8(+) T cell-mediated disease. Moreover, this method may be helpful for the differential diagnosis of polymyositis and dermatomyositis and for noninvasive follow-up of polymyositis patients.

Adult↗
PubMedDate not supplied

Usefulness of erythrocyte sedimentation rate as tumor marker in cancer associated dermatomyositis.

Dermatomyositis is an inflammatory myopathy characterized by proximal symmetrical muscle weakness with a characteristic cutaneous eruption. Population-based cohort studies provide evidence of an increased frequency of cancer in dermatomyositis patients. Many signs and serology tests have been suggested as markers for malignancy in dermatomyositis. We performed a case-control study on the patients admitted in our institutions for dermatomyositis. Clinical and laboratory data were collected and statistical analysis was performed to reveal important predictive signs of malignancy in dermatomyositis. We found no statistical difference in the clinical or laboratory parameters between the dermatomyositis patients with or without malignancy, with the exception of the erythrocyte sedimentation rate. In our study group an erythrocyte sedimentation rate higher than 35 mm/hr was very strongly associated with the presence or the development of a malignancy. Given the high positive and negative predictive values observed in our study, erythrocyte sedimentation rate evaluation using this cut-off point could be very useful in alerting dermatologists to the need for more in-depth diagnostic procedures in dermatomyositis patients.

Blood Sedimentation↗
PubMed2001

[Dermatomyositis and polymyositis: clinical aspects and treatment].

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Long-term prognosis of 69 patients with dermatomyositis or polymyositis.

OBJECTIVES: To assess the long-term prognosis of dermatomyositis and pol myositis. METHODS: 69 patients with dermatomyositis or polymyositis were selected according to the diagnostic criteria of Bohan and Peter and were followed up for a minimum of 6.3 years (for surviving patients) (mean 11.6 years). Clinical and biological features, and pulmonary and muscle parameters were considered as prognostic factors for death. Functional disability was assessed using a 4-stage grading system. RESULTS: 30 deaths (43.5%) occurred mainly due to cardiovascular (8), pulmonary (8), carcinomatous (5) and iatrogenic complications (5). Survival rates were 82.6% at 1 year, 73.9% at 2.66, 7% at 5 and 55.4% at 9. Significant prognostic factors for death (Cox model with time-dependent covariates) were old age (p < 0.0001), dysphonia (p < 0.001), pulmonary interstitial fibrosis (p < 0.02), absence of dysphagia (p < 0.02) and asthenia-anorexia (p < 0.05). Dermatomyositis and polymyositis subgroups had slightly different significant prognostic factors for death: old age, cancer, pulmonary interstitial fibrosis and asthenia-anorexia for dermatomyositis; old age, failure to improve muscle strength in response to treatment after one month, and the absence of myalgia as presenting symptom for polymyositis. At the end of the follow-up, 33/39 surviving patients (84.6%) had no or insignificant muscular disability, whereas 3 children were bedridden due to generalized calcinosis. CONCLUSIONS: High mortality occurred in the first year, and the survival rate decreased continually up to 9 years. The main prognostic factor for death is old age, but dermatomyositis and polymyositis must be considered separately. General features (pulmonary fibrosis, cancer, asthenia-anorexia) are involved in dermatomyositis, whereas muscular symptoms are the most significant in polymyositis. The long-term functional prognosis was fairly good, except for generalized calcinosis, which tended to occur in childhood dermatomyositis.

Adolescent↗
PubMed2003

Fasciitis in amyopathic dermatomyositis.

Dermatomyositis is a disease frequently treated by rheumatologists and dermatologists due to prominent systemic features of inflammatory myositis, less common arthritis, and rare systemic vasculitis, in addition to the characteristic cutaneous manifestations of Gottron's papules over extensor surfaces, and a heliotrope rash over the eyelids. Patients with amyopathic dermatomyositis, a subset of dermatomyositis, display skin disease but no apparent muscle disease. This report describes an adult patient with the typical dermatomyositis rash with no weakness, normal muscle enzymes, and the unique finding of fasciitis without myositis on muscle biopsy, which correlated with a Magnetic Resonance Imaging (MRI) finding of a peripheral halo of intense signal around muscles on T2-weighted and fat suppression sequences. Although MRI finding of presumed fasciitis has been reported in juvenile-onset dermatomyositis, this is the first report of MRI evidence of fasciitis with pathological correlation in adult-onset dermatomyositis. We suggest that if MRI scans are ordered, as part of the work-up of dermatomyositis, a peripheral halo of increased signal should specifically be looked for, which could be interpreted as fasciitis based on this case report.

Journal Article↗
PubMed2002

Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology.

OBJECTIVE: To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen patients with amyopathic dermatomyositis. RESULTS: The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one. CONCLUSIONS: Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.

Adult↗
PubMed2005

Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis.

Dermatomyositis has been modeled as an autoimmune disease largely mediated by the adaptive immune system, including a local humorally mediated response with B and T helper cell muscle infiltration, antibody and complement-mediated injury of capillaries, and perifascicular atrophy of muscle fibers caused by ischemia. To further understand the pathophysiology of dermatomyositis, we used microarrays, computational methods, immunohistochemistry and electron microscopy to study muscle specimens from 67 patients, 54 with inflammatory myopathies, 14 with dermatomyositis. In dermatomyositis, genes induced by interferon-alpha/beta were highly overexpressed, and immunohistochemistry for the interferon-alpha/beta inducible protein MxA showed dense staining of perifascicular, and, sometimes all myofibers in 8/14 patients and on capillaries in 13/14 patients. Of 36 patients with other inflammatory myopathies, 1 patient had faint MxA staining of myofibers and 3 of capillaries. Plasmacytoid dendritic cells, potent CD4+ cellular sources of interferon-alpha, are present in substantial numbers in dermatomyositis and may account for most of the cells previously identified as T helper cells. In addition to an adaptive immune response, an innate immune response characterized by plasmacytoid dendritic cell infiltration and interferon-alpha/beta inducible gene and protein expression may be an important part of the pathogenesis of dermatomyositis, as it appears to be in systemic lupus erythematosus.

Adult↗
PubMed1999

Dermatomyositis and drugs.

Dermatomyositis (DM) is an idiopathic inflammatory disorder consisting of skin and skeletal muscle involvement. Patients with skeletal muscle involvement have polymyositis (PM), and those unresponsive to therapy and with characteristic findings on muscle biopsy have inclusion body myositis. Patients without muscle damage and typical skin lesions have amyopathic dermatomyositis. Disease in children (juvenile dermatomyositis) is not associated with malignancy as it may be in adults (paraneoplastic dermatomyositis). Overlap syndrome (OS) is mixed connective tissue disease combining some features of DM, SS and LES. Scleromyositis is overlap syndrome associated with anti-PM-Sci antibodies. Patients with PM, DM or OS with "interstitial lung disease" and anti-synthetase antibodies have an "anti-synthetase syndrome". Various drugs, including d-penicillamine, NSAIDs, anti-infectious agents, as well as lipid lowering drugs, the HMG-CoA reductase inhibitors may cause myopathy and skin lesions (drug induced dermatomyositis). "Dermatomyositis" occurring as adverse reactions of drugs are rare, irregular and impossible to predict in individual patients. They are very interesting in that they may be keys for explaining the pathogenic mechanisms of the disease.

Dermatomyositis↗
PubMed1999

Dermatomyositis associated with malignancy. 12 case reports.

Dermatomyositis (DM) is an idiopathic inflammatory disease of unknown etiology which manifests itself with symmetrical proximal muscle weakness and typical heliotrope skin rash. Internal malignancy is a specific trigger for some cases of DM. The aim of this study is to find predictive signs of cancer in dermatomyositis patients. Twelve (2 males and 10 females) (24%) out of 50 adult patients with DM (10 males and 40 females between 21 and 81 years old) had associated with malignancy (paraneopastic dermatomyositis, PDM). The male: female ratio in patients with idiopathic dermatomyositis (IDM) was 1:3.75 and for PDM it was 1:5. The frequency of dermatomyositis increases with age. PDM is most frequent between 40 and 69 years of life. Associated malignancies were respiratory tract carcinomas in males, genital and breast carcinomas in females. DM preceded the carcinoma in 5 cases (41.7%); 7 patients (58.3%) had already been treated for malignancy. In conclusion PDM in our country is more frequent in females and in the age after 40 years. Association of dermatomyositis with malignancy is relatively high.

Adult↗
PubMed2005

Preservation of in vitro muscle fiber function in dermatomyositis and inclusion body myositis: a single fiber study.

Five patients with untreated dermatomyositis, five with inclusion body myositis, and 16 healthy elderly volunteer subjects (controls) underwent open (dermatomyositis and inclusion body myositis) or percutaneous (controls) muscle biopsy. Biopsied muscles included deltoid, biceps and vastus lateralis. Chemically skinned single muscle fibers were activated with Ca(+2); the slack test was performed to determine maximal unloaded shortening velocity (Vo). Parameters measured include single fiber cross sectional area, maximal force, specific force and Vo. 429 Type I and 94 Type IIA fibers were studied. Cross sectional area and maximal force were greater in inclusion body myositis than dermatomyositis or control for Type I and IIA fibers. Specific force of Type I fibers was similar in inclusion body myositis and dermatomyositis but greater than in controls. Vo was greater in Type I, but not IIA, fibers in dermatomyositis compared with inclusion body myositis and controls. The force and velocity generating capacity of single muscle fibers is preserved in patients with dermatomyositis and inclusion body myositis suggesting that dysfunction of the contractile proteins does not contribute to clinical muscle weakness.

Adult↗
PubMed1998

Dermatomyositis: a dermatology-based case series.

BACKGROUND: Dermatomyositis is associated with significant morbidity and occasional mortality. Currently there is no consensus on treatment for patients with dermatomyositis. OBJECTIVE: Our purpose was to review the clinical features and response to therapy of patients with dermatomyositis and compare these data with previous series of patients with dermatomyositis/polymyositis. METHODS: Clinical characteristics of 65 patients seen during a 10-year period were reviewed retrospectively. Twenty-one of these patients were enrolled in a prospective, uncontrolled study of treatment with high-dose prednisone followed by slow tapering. RESULTS: Clinical features were similar to those previously described; however, muscle strength at diagnosis was on average greater in patients in this series than in patients previously reported. Malignancy was present in 5 of 43 adult patients (12%), but was not found in patients with juvenile dermatomyositis. Another connective tissue disease was present in 19% of patients. Twelve patients had dermatomyositis sine myositis. Eighteen of 21 patients (85%) in the prednisone study group had resolution of myositis. CONCLUSION: Patients with dermatomyositis in this series had less active myositis at presentation, but were otherwise similar to patients with dermatomyositis/polymyositis previously reported. Treatment with high-dose daily prednisone followed by slow tapering was effective.

Adult↗
PubMed2002

Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study.

BACKGROUND: Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear. PATIENTS AND METHOD: During the 30-year period 1969-99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer. RESULTS: The risk of cancer is significantly higher in dermatomyositis and somewhat higher in polymyositis. An increased cancer risk was found in male patients with dermatomyositis who were older than 50 years. Cancer was diagnosed within 4 years before or after the diagnosis of polymyositis or dermatomyositis, and usually within 1 year. An operation was not possible in many of the patients with cancer because of the advanced stage of the disease. CONCLUSION: Our findings suggest that early discovery of malignancy is critical in cases of polymyositis and dermatomyositis.

Adult↗
PubMedDate not supplied

Panniculitis in juvenile dermatomyositis.

Panniculitis is a rarely reported clinical finding in dermatomyositis. We present a 14-year-old African American boy with a 4-year history of dermatomyositis referred for evaluation of tender, indurated plaques and nodules on the trunk and proximal extremities. A biopsy specimen revealed epidermal and dermal changes consistent with dermatomyositis. Although calcification was absent, a striking lobular panniculitis was observed. A total of seven cases of clinical panniculitis in association with dermatomyositis have been published. As in our patient, it typically presents as indurated, tender plaques and nodules on the arms, thighs, and buttocks. Although in the vast majority of cases panniculitis found in association with dermatomyositis is subclinical and represents an incidental histopathologic finding, the case we present further emphasizes the need to recognize panniculitis as a rare, but clinically relevant pathologic feature seen in certain lesions of juvenile dermatomyositis.

Adolescent↗
PubMed2002

Amyopathic dermatomyositis: retrospective review of 37 cases.

Criteria for diagnosis of amyopathic dermatomyositis vary, and the prognosis is not clear. Our purpose was to investigate prognosis regarding progression to myositis and associated malignancy. We reviewed the medical records of patients with dermatomyositis evaluated at our institution from 1976 to 1994. Of 746 patients with dermatomyositis, 37 (5%) with the amyopathic subtype were divided into 3 groups: group 1 (73%), no subjective or objective evidence of myopathy; group 2 (13%), no subjective muscle weakness but abnormalities detected by objective tests; group 3 (13%), subjective muscle weakness but no objective evidence of myopathy. Follow-up was conducted by means of a mailed questionnaire. For 25 patients, follow-up of 1 to 17 years after diagnosis showed muscle weakness in 2 patients in group 1 within 5 years after diagnosis. Five patients (13%) had associated malignancies. Of 7 (19%) patients with disease onset before the age of 18 years, none had progression to myopathy. Although it presents with cutaneous lesions indistinguishable from those of classic dermatomyositis, amyopathic dermatomyositis is a distinct entity. In most patients, amyopathic dermatomyositis does not progress to myopathy. Prognosis appears favorable, but malignancy may develop.

Adolescent↗
PubMed2002

Clinical assessment of conjunctival and episcleral vessel tortuosity in juvenile dermatomyositis.

PURPOSE: Conjunctival and episcleral vessel tortuosity are thought to be associated features of juvenile dermatomyositis. This study was conducted to determine the interobserver reliability of assessing normal and abnormal conjunctival and episcleral vessels from photographs of patients with and without juvenile dermatomyositis. METHODS: Color and red-free external ocular photographs of each eye of 28 children were assessed by 5 pediatric ophthalmologists from The Hospital for Sick Children. Fifteen of these pairs of photographs were of normal control eyes and 13 were from eyes of patients with juvenile dermatomyositis. On a standardized form each ophthalmologist was asked to determine whether both the conjunctival and episcleral vessels appeared normal or abnormal. Interobserver agreement was calculated by the weighted kappa statistic. The sensitivity and specificity of abnormal vessels for diagnosing juvenile dermatomyositis were also determined. RESULTS: Interobserver agreement for assessing conjunctival and episcleral vessels was 0.18 and -0.005, respectively. The average sensitivity and specificity of the evaluators for correctly assessing conjunctival vessels were 0.70 and 0.70, and for episcleral vessels, 0.67 and 0.47, respectively. CONCLUSIONS: There was low interobserver agreement in distinguishing between normal and abnormal eyes based on conjunctival and episcleral vessels. The sensitivity and specificity for identifying patients with juvenile dermatomyositis based on the appearance of vessels alone were relatively low. The appearance of conjunctival and episcleral vessels should be assessed in the context of other ocular and periocular findings of patients with juvenile dermatomyositis.

Adolescent↗
PubMed1999

MR imaging in amyopathic dermatomyositis.

PURPOSE: Amyopathic dermatomyositis is a distinct clinical entity with cutaneous involvement but no myopathy. We conducted a prospective study to investigate the role of MR imaging in these patients. MATERIAL AND METHODS: Out of 40 Chinese patients presenting with dermatomyositis, based on clinical assessment and normal serum muscle enzymes 10 were diagnosed as having amyopathic dermatomyositis. These 10 patients underwent MR imaging for evaluation of any subclinical muscle involvement. RESULTS: Three patients demonstrated abnormal signal intensity in muscles on both T2- and fat suppression sequences. Thus, one-third of patients with dermatomyositis and clinically normal muscles may have detectable muscle inflammation on MR images, indicating that MR has a potential role for locating the relevant biopsy site and for longitudinal follow up. Six of the 10 patients had malignant disease diagnosed before or after diagnosis of the cutaneous manifestation. Nasopharyngeal carcinoma was the most common malignant disease in this group of patients. CONCLUSION: MR imaging is recommended for demonstrating subclinical muscle involvement in patients with the clinical diagnosis of amyopathic dermatomyositis. We also recommend screening for malignancy, particularly nasopharyngeal carcinoma, in Southern Chinese patients with dermatomyositis.

Adult↗