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PubMed2005

Cutaneous symptoms of dermatomyositis significantly impact patients' quality of life.

BACKGROUND: Dermatomyositis affects visible skin and causes disease symptoms that can affect patients' quality of life (QOL). METHODS: In all, 71 patients with dermatomyositis or dermatomyositis sine myositis completed two QOL measures (the Skindex-16 and the Dermatology Life Quality Index) and a visual analog scale for pruritus. Disease severity was assessed by Physician's Global Assessment. RESULTS: The mean Dermatology Life Quality Index score was 10.7 and the mean Skindex-16 score was 51.1. Itching contributed to impact on both the Dermatology Life Quality Index and Skindex-16. Females reported worse QOL. LIMITATIONS: The effect of treatment on quality of life was not assessed in these analyses. CONCLUSION: QOL impairment in dermatomyositis is greater than in other skin conditions including psoriasis and atopic dermatitis. Pruritus is an important treatable factor that significantly impacts QOL for patients with dermatomyositis.

Adolescent↗
PubMed1999

Paraneoplastic amyopathic dermatomyositis associated with breast cancer recurrence.

Amyopathic dermatomyositis, also known as dermatomyositis sine myositis, describes patients with the typical cutaneous features of dermatomyositis but without evidence of inflammatory myopathy. There have been 3 reported cases of amyopathic dermatomyositis associated with breast cancer in patients with no prior malignancy. We describe the first case of paraneoplastic amyopathic dermatomyositis associated with breast cancer recurrence.

Adenocarcinoma↗
PubMed1998

Treacher-Collins syndrome and co-existing dermatomyositis.

BACKGROUND: Treacher-Collins syndrome, an autosomal dominantly inherited malformation of structures derived from the first and second branchial arch, has an incidence of 1:10,000 newborns. The prevalence of dermatomyositis at less than 24 years of age has been estimated at 1 per 100,000. The occurrence of both Treacher-Collins syndrome and dermatomyositis combined in the same patient should occur once in every 1,000,000,000 subjects. METHODS: We report a patient with Treacher-Collins syndrome who developed dermatomyositis at the age of 5 years. RESULTS: No other patient with both Treacher-Collins syndrome and an autoimmune disease has been reported. The thymus originates from the third branchial pouch and is unaffected by the syndrome. In Treacher-Collins syndrome the affected gene has been mapped to the fifth chromosome, while dermatomyositis is related to HLA B8 and DR3, coded on the sixth chromosome. No immunologic alteration has been described in patients with Treacher-Collins syndrome. CONCLUSION: This is the first report of a patient with Treacher-Collins syndrome and dermatomyositis. There is no genetic or physiopathologic explanation for the concurrence of both conditions.

Anti-Inflammatory Agents↗
PubMed2002

Dermatomyositis sine myositis and antisynthetase syndrome.

We describe a 66-year-old woman with cutaneous lesions typical of classic dermatomyositis, clinical evidence of antisynthetase syndrome (arthritis, Raynaud's phenomenon, mechanic's hands and interstitial lung disease with anti-Jo-1 autoantibody) and lack of muscle disease after a full muscle evaluation that included clinical, enzymatic, electromyographic, magnetic resonance imaging and histological studies. The patient did not develop myositis after 9 years of clinical disease. The association of dermatomyositis sine myositis with antisynthetase antibodies suggests that the characteristic skin lesions are closely linked with dermatomyositis on the basis of the similar clinical and serological features of both dermatomyositis sine myositis and classic dermatomyositis.

Aged↗
PubMed1997

Malignancy in adult dermatomyositis.

BACKGROUND: Dermatomyositis has been reported to be associated with malignancies in 15%-34% of patients in Western countries, but in as many as two-thirds of patients in Singapore. The aim of this study was to determine whether a diagnostic measure could be helpful in the diagnosis of a malignancy in patients with dermatomyositis. METHODS: This is a retrospective study on 38 adult patients with dermatomyositis that were seen over a 6-year period from 1989 to 1994. RESULTS: All the patients presented with cutaneous features that suggest the clinical diagnosis of dermatomyositis; however, not all cases show all the key features of the disease. Of the studied patients, 86.8% were noted to have photosensitivity as a key cutaneous presentation. Thirty (78.9%) of our patients were above the age of 40 years, and 12 (31.6%) of these were found to have an associated malignancy. Nasopharyngeal carcinoma was the most commonly associated malignancy (38.4%) in our study population. CONCLUSIONS: In our study population, otorhinolaryngologic screening is an essential investigation for the evaluation of dermatomyositis in association with malignancy.

Adolescent↗
PubMed1986

Microvascular deposition of complement membrane attack complex in dermatomyositis.

We examined the role of the complement system in the pathogenesis of dermatomyositis. Using an antibody against the neoantigens of the terminal C5b-9 membrane attack complex, we performed immunocytochemical studies that localized this complex to the intramuscular microvasculature (arterioles and capillaries) of muscle biopsy specimens from 10 of 12 patients (83 percent) with childhood dermatomyositis and 5 of 19 patients (26 percent) with adult dermatomyositis. Fifty-two control specimens, including 14 from patients with polymyositis and 12 from patients with denervation atrophy (a condition known to be associated with necrotic capillaries), showed no deposition of membrane attack complex in the microvasculature. These findings indicate that the complement system is deposited, bound, and activated to completion within the intramuscular microvasculature of patients with dermatomyositis. In addition to providing further evidence for the presence of vasculopathy in dermatomyositis, these findings suggest a primary role for complement in mediating vessel injury in the disease, particularly in its childhood form.

Adult↗
PubMed1994

Dermatomyositis associated with Lyme disease: case report and review of Lyme myositis.

Myositis due to the etiologic agent of Lyme disease, Borrelia burgdorferi, has been reported nine times in the English-language literature; there has been but a single report of exacerbation of dermatomyositis due to B. burgdorferi in a patient with known dermatomyositis. Multiple infectious agents, but not B. burgdorferi, have been hypothesized to trigger dermatomyositis. We report the first case of dermatomyositis that appears to have been triggered by B. burgdorferi. This case involved an individual from Westchester County, NY, who presented with skin lesions suggestive of erythema migrans and who was seropositive for Lyme disease. He soon developed a clinical syndrome suggestive of dermatomyositis: periorbital edema, dysphagia, proximal muscle weakness, and a markedly elevated level of creatine phosphokinase. We also review the clinical presentation and response to treatment of patients with Lyme myositis.

Anti-Bacterial Agents↗
PubMed2004

Uncertainties in the pathogenesis of adult dermatomyositis.

PURPOSE OF REVIEW: Dermatomyositis is an inflammatory disorder of muscle affecting both children and adults. The pathogenesis of adult dermatomyositis is reviewed here, with particular attention to gaps and uncertainties in our current understanding of this disease, in order to focus attention on the outstanding research questions with regard to disease mechanisms. RECENT FINDINGS: The conceptual model of the pathophysiology of dermatomyositis has been based on work extending back over at least 35 years, with important findings related to the deposition of membrane attack complex, the character of the inflammatory infiltrate, and the primacy of capillary abnormalities established over 10 years ago. The lack of significant additions to the understanding of this disease over the past few years suggests a state of contentment with current knowledge. Accordingly, we emphasize the uncertainties of this knowledge and the need for re-addressing some of its basic tenets. SUMMARY: Although evidence remains strong that dermatomyositis is a disorder with an early involvement of the capillaries, the concept that it results from an antibody-mediated attack upon the endothelium is uncertain. It is not clear whether membrane attack complex is activated by antibody-dependent or independent means, whether or not intramuscular B cells are antigen specific and synthesize significant quantities of antibodies, whether the presence of tubuloreticular inclusions precedes, follows, or is independent of membrane attack complex deposition, what is the mechanism of perifascicular atrophy, and what is the relationship of dermatomyositis to systemic lupus erythematosus.

Adult↗
PubMed2005

Interstitial lung disease in polymyositis and dermatomyositis.

PURPOSE OF REVIEW: The purpose of this review is to discuss current concepts regarding management of interstitial lung disease in polymyositis and dermatomyositis. RECENT FINDINGS: Interstitial lung disease seems to be a more frequent manifestation in patients with polymyositis and dermatomyositis than previously reported. Modern technology, including high-resolution computerized tomography in combination with pulmonary function tests provides sensitive tools to detect early signs of interstitial lung disease. By systematic use of these investigations in newly diagnosed polymyositis and dermatomyositis, up to two thirds of patients were discovered to have signs of interstitial lung disease in a recent study. Clinical symptoms such as cough and dyspnea may not be sensitive enough to detect interstitial lung disease. Awareness of this complication in patients with myositis is important, because early diagnosis and management of interstitial lung disease may prevent development of chronic pulmonary fibrosis and thereby prolong patient survival and improve quality of life. Treatment recommendations of interstitial lung disease in polymyositis and dermatomyositis are still limited by absence of controlled trials and could only be based on experiences from small case series and case reports. At least some patients with interstitial lung disease improve with immunosuppressive treatment, but data are limited, and longitudinal studies are needed. SUMMARY: Interstitial lung disease seems to be a common manifestation in patients with polymyositis and dermatomyositis already at diagnosis of the muscle disease. When present, interstitial lung disease has a major effect on morbidity and mortality and should be looked for in these patients using high-resolution computerized tomography and pulmonary function tests early in the disease course, because immunosuppressive treatment may change the course of the lung disease.

Biopsy↗
PubMed2006

Advances in the treatment of juvenile dermatomyositis.

PURPOSE OF REVIEW: Juvenile dermatomyositis is a rare chronic inflammatory disease that primarily affects the muscles and skin. Immunosuppressive therapy has played a very important role in reducing mortality rates and morbidity. The review focuses on the spectrum of medications currently used in the treatment of juvenile dermatomyositis, highlighting new advances and unanswered questions. RECENT FINDINGS: Data regarding the treatment of juvenile dermatomyositis come almost entirely from retrospective studies with relatively small numbers of patients. Corticosteroids continue to be the accepted first-line therapy. Evidence that the addition of methotrexate at initiation of treatment allows corticosteroids to be tapered more rapidly with good outcomes exists. High-risk, refractory patients may benefit from intravenous cyclophosphamide. Results in refractory patients treated with rituximab are also encouraging. Topical immunosuppressant agents have been largely disappointing in treating rash. The effect and role of exercise in the treatment and rehabilitation of patients with juvenile dermatomyositis is an interesting new area of research. SUMMARY: Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity for the individual patient.

Child↗
PubMed2006

Dermatomyositis and esophageal cancer.

OBJECTIVE: A case of dermatomyositis and esophageal cancer is described. CASE REPORT: A 58-year-old male recently diagnosed with esophageal cancer was admitted to the hospital with complaints of progressive dysphagia, generalized muscle weakness and skin rash. The weakness started symmetrically in the proximal limb muscles. He also developed a characteristic skin rash on the eyelids, the upper chest and around the nails that was thought to be most indicative of dermatomyositis. Creatine kinase, aspartate aminotransferase and aldolase were elevated. A muscle biopsy showed various degrees of degeneration with perivascular interstitial infiltration of lymphoplasma cells, a finding consistent with "dermatomyositis." The patient was started on corticosteroids and within two weeks, his muscle strength was found to be markedly improved and the rash almost disappeared. CONCLUSION: Dysphagia can be the presenting symptom of both dermatomyositis and esophageal cancer. In the setting of an underlying malignancy, these symptoms can be misleading and one can miss the diagnosis of dermatomyositis. However, recognition of the characteristic skin rash may provide a clue to the diagnosis. Another aspect of our case that is worth acknowledgment is the quick response to treatment with corticosteroids.

Adenocarcinoma↗
PubMed1989

Carcinoma-associated dermatomyositis responding to plasmapheresis.

We report a case of severe carcinoma-associated dermatomyositis which, having shown no response to conventional treatment, improved markedly within 48 h of plasmapheresis. We believe that this is the first time that dermatomyositis associated with an advanced carcinoma has been reported to respond in this way. Dermatomyositis can prove extremely difficult to treat and sometimes fails to respond even to very high doses of steroids and immunosuppressants. There is currently only a single report of the use of plasmapheresis in adult dermatomyositis. We therefore wish to support the findings of Dau and draw attention to the value of this therapeutic option in refractory dermatomyositis.

Adenocarcinoma↗
PubMed2006

Neuroendocrine carcinoma of the liver associated with dermatomyositis: autopsy case and review of the literature.

Reported herein is an autopsy case of primary hepatic neuroendocrine carcinoma associated with dermatomyositis. A 71-year-old Japanese man, who was diagnosed with dermatomyositis 5 months before death, had multiple tumors within a non-cirrhotic liver. Histopathologically, the tumors were composed of small- and medium-sized round cells with clear cytoplasm arranged in nests, sheets or rosettes. Immunohistochemically, the tumor cells were positive for chromogranin A, neuron-specific enolase and CD56 and were negative for synaptophysin. This tumor was diagnosed as a primary hepatic neuroendocrine carcinoma with metastasis to the lung, gallbladder and lymph nodes around the pancreas and aorta; no primary lesions were detected in any other organ. The tumor cells were also positive for cytokeratin 7, cytokeratin 19 and epithelial membrane antigen but were negative for anti-hepatocyte antibody and AFP. These findings suggest that the tumor originated in intrahepatic bile duct epithelium. Various cancers have been reported in patients with dermatomyositis, but only seven cases of dermatomyositis associated with primary liver cancer have been reported. To the best of the authors' knowledge, this is the first report of dermatomyositis associated with primary hepatic neuroendocrine carcinoma.

Aged↗
PubMed2004

[A case of dermatomyositis associated with different types of cancers at intervals of six years].

The patient is a 56-year-old Japanese woman who suffered from breast cancer and ovarian cancer at intervals of 6 years, and was also complicated by two episodes of dermatomyositis, each of which occurred simultaneously with each of two cancers. When she was 51 years old, she developed dermatomyositis for the first time 6 months after the resection of breast cancer, whose histological type was tubular adenocarcinoma. The dermatomyositis remitted without oral corticosteroids in 2 months, and the remission had continued for 6 years. However, at the age of 56, dermatomyositis abruptly recurred with a pruritic generalized rash, Gottron's papules and elevated serum CK levels. Examination for malignancy revealed an ovarian tumor, which was diagnosed as serous papillaly adenocarcinoma, and the surgery was performed. After the resection of the ovarian cancer, skin rash was improved dramatically and CK levels were normalized again without oral corticosteroids. Since there were no evidences of recurrence of the breast cancer, it was considered that each episode of dermatomyositis was associated with each of the cancers, respectively. We report this rare and interesting case to consider the etiology of cancer-associated myositis as a paraneoplastic syndrome, since the two cancers have different histological types.

Adenocarcinoma↗
PubMed1993

MR imaging in children with dermatomyositis: musculoskeletal findings and correlation with clinical and laboratory findings.

OBJECTIVE: The purpose of this study was to describe the musculoskeletal MR findings in childhood dermatomyositis and to correlate MR findings with indicators of disease activity such as muscle strength and serum levels of muscle enzymes. SUBJECTS AND METHODS: This prospective study included 24 children: 19 children with dermatomyositis and five control subjects. The diagnosis of dermatomyositis was established by clinical findings and serum levels of muscle enzymes in all patients, electromyography in six patients, and biopsy in four patients. At the time of the initial MR evaluation, patients were classified on the basis of clinical findings as having active (n = 15) or inactive (n = 4) disease. A total of 44 MR evaluations of patients with dermatomyositis were included in the study: 19 initial MR examinations and 12 examinations repeated after 4-6 months of therapy in patients with active disease. An additional 13 examinations were performed on five patients. Conventional T1-weighted (SE 600/20) and T2-weighted (SE 2500/80) spin-echo and fat-suppressed MR images were obtained. The T2-weighted images (TE = 80) were used for comparison. In addition to the visual assessment, ratios between the signal intensity of muscles (gluteus, adductors, quadriceps, and hamstrings) and the signal intensity of subcutaneous fat in the same tomographic section were calculated. RESULTS: All patients with clinically active disease (n = 15) had abnormal findings on MR studies, whereas those with inactive disease (n = 4) had normal MR findings. Signal-intensity ratios of patients with active disease were greater than those in control subjects, whereas the ratios in patients with inactive disease were not different from those in control subjects. After 4-6 months of therapy, the average signal-intensity ratios of treated patients with repeated MR evaluations (n = 12) differed from ratios obtained before therapy in the same patients, but were not different from the ratios in control subjects. Other MR findings observed were perimuscular edema, enhancement of the chemical-shift artifact, and inflammatory changes of subcutaneous fat. Fat-suppressed imaging enhanced visualization of abnormalities. Markedly abnormal signal intensities of muscle were associated with marked elevations of serum levels of muscle enzymes; however, abnormal MR findings were visualized with normal serum levels of muscle enzymes. CONCLUSION: Findings of active childhood dermatomyositis on T2-weighted MR images include increased signal intensity in affected muscle, perimuscular edema, enhanced chemical-shift artifact, and increased signal intensity in subcutaneous fat. After therapy, signal intensity of muscle returns to normal. These MR findings are enhanced on fat-suppressed images.(ABSTRACT TRUNCATED AT 400 WORDS)

Adipose Tissue↗
PubMed2000

Dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia.

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.

Azathioprine↗
PubMed2000

[Dermatomyositis and small-cell lung cancer : fortuitous association or paraneoplastic syndrome?].

Dermatomyositis, an inflammatory muscle disease probably related to dysimmunity, is associated with character skin eruptions. Dermatomyositis is often associated with cancer (15 to 40% of cases depending on the series). All histological types and all cancer localizations observed in the general population can be associated with dermatomyositis. We report the case of a patient with dermatomyositis who developed small-cell lung cancer. There have been few descriptions of this association in the literature. Certain clinical features of dermatomyositis would be predictive of its paraneoplastic nature. Prognosis is very poor. Treatment is basically dictated by the underlying neoplasia.

Aged↗
PubMed2000

Classical and amyopathic dermatomyositis seen at the National Skin Centre of Singapore: a 3-year retrospective review of their clinical characteristics and association with malignancy.

INTRODUCTION: Dermatomyositis is a condition well-known to medical physicians, especially rheumatologists and dermatologists. Classically, patients present with typical cutaneous features and proximal muscle weakness. Amyopathic dermatomyositis has been reported in recent literature where patients present with skin changes without muscle involvement. MATERIALS AND METHODS: We reviewed 28 patients diagnosed with dermatomyositis at the National Skin Centre over a 3-year period from 1996 to 1998 to assess the prevalence of this amyopathic variant in our local population and its association with malignancy. RESULTS: Out of the 28 patients, 13 (46.4%) had no clinical or laboratory evidence of myositis at presentation. Nine patients (32.1%) had clinical muscle weakness and 6 patients (21.4%) had laboratory evidence of myositis. Malignancies were detected in 12 patients (42.8%), half of which were nasopharyngeal carcinomas. There was no significant difference in the prevalence of malignancy between those with detectable muscle weakness (33.3%) and those without (47.4%). However, there was a significant higher prevalence of malignancy in those with clinical and laboratory evidence of myositis (66.6%) than those without (15.4%). CONCLUSION: We conclude that amyopathic dermatomyositis is a common presentation in our population and may have a lower risk of malignancy than the classical variant. Screening for malignancy, especially nasopharyngeal carcinoma, is recommended for all dermatomyositis patients.

Adolescent↗