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PubMed2004

Dermatomyositis associated with autoimmune idiopathic thrombocytopenia and anti-Ku antibody.

We describe a case of dermatomyositis with concurrent clinical and laboratory features of idiopathic thrombocytopenia associated with anti-Ku antibody. A diagnosis of dermatomyositis was established by the characteristic skin changes together with a muscle biopsy. Scintigraphic studies indicated cardiac involvement. Autoimmune idiopathic thrombocytopenia (AITP) has been described in association with both systemic lupus erythematosus (SLE) and scleroderma, but there are few reports describing AITP associated with myositis. To our knowledge, this is the first report of a case of dermatomyositis associated with AITP and anti-Ku antibody.

Journal Article↗
PubMed2001

Amyopathic dermatomyositis in children: a diagnostic and therapeutic dilemma.

Juvenile dermatomyositis is an inflammatory disease of unknown etiology that primarily affects skin and muscles. The pathognomonic Gottron's sign consists of symmetric macules and papules on the dorsal aspect of the interphalangeal joints and exterior areas of the big joints. A periorbital violaceous (heliotrope) skin rash is also characteristic. There may be a discordance in time of presentation of the skin and muscle disease, and a small subset of patients apparently do not develop muscle disease at all. The absence of muscle involvement is termed 'amyopathic dermatomyositis.'We describe two children who presented with the characteristic rash of juvenile dermatomyositis but with no clinical evidence of muscle involvement. One developed muscle weakness 3 years later. Neither patient had a full muscle work-up at the onset of the disease, which left questions about diagnoses and whether or not there may have been subtle muscle involvement. On the basis of our literature review, the outcome of these patients is uncertain, although it appears that myositis develops in many, maybe most, affected children. We suggest that in the absence of muscle disease, application of sunscreen and administration of hydroxychloroquine sulfate may ameliorate the rash. More aggressive treatment will need to be given when muscle involvement can be demonstrated.

Journal Article↗
PubMed2004

MHC Class I overexpression on muscles in early juvenile dermatomyositis.

OBJECTIVE: To assess muscle expression of MHC Class I complexes (heavy chain and beta2-microglobulin) and to analyze the composition of infiltrating mononuclear cells, specifically cells that bear receptors for class I MHC molecules, in the muscles of children with early juvenile dermatomyositis (JDM). METHODS: Light microscopic and immunohistochemical analysis of muscle biopsies from 10 patients with JDM and 3 controls. The mean duration from initial weakness was 2.8 months. At the time of biopsy, 9 patients had not received steroid treatment or immunomodulatory drugs. RESULTS: MHC Class I over-expression was evident on muscle fibers in all 10 JDM samples, even in a biopsy reported as normal by conventional histology. MHC class I heavy chain and beta2-microglobulin were over-expressed in an identical distribution. Variable infiltration of T cells and macrophages was seen in the JDM biopsies, with minimal lymphocytic and monocytic infiltration in 4 cases, and none in one. Only very occasional natural killer lymphocytes were identified. Neuronal cell adhesion molecule (NCAM, CD56) staining of regenerating muscle fibers was seen in all samples and these cells were confirmed as being of muscle origin by co-staining for dystrophin. CONCLUSION: MHC Class I over-expression is an early event in JDM, and may occur in the absence of lymphocytic infiltration and muscle damage. Immunostaining for MHC Class I could be used routinely in the assessment of muscle histology in juvenile dermatomyositis.

Adolescent↗
PubMed2005

Combination antimalarials in the treatment of cutaneous dermatomyositis: a retrospective study.

OBJECTIVE: To observe whether the use of antimalarials in combination resulted in significant improvement in the cutaneous signs and symptoms of patients with dermatomyositis who did not otherwise respond to the use of single-agent antimalarial therapy. DESIGN: Retrospective case series of 17 patients treated between January 1, 1991, and December 31, 2002. SETTING: An ambulatory medical dermatology clinic in an academic center.Patients Patients had adult-onset dermatomyositis with predominantly cutaneous symptoms and a follow-up period at our clinic of at least 6 months. Cases in which it was not possible to assess the effect of treatment on cutaneous symptoms were not included. Intervention Treatment regimens varied and included the use of antimalarials, prednisone, methotrexate, and other medications. MAIN OUTCOME MEASURES: Physician-observed and patient-reported improvement based on erythema, pruritus, and extent of affected skin. RESULTS: Seven of 17 patients experienced at least near clearance in cutaneous symptoms with the use of antimalarial therapy alone: 4 of these patients required combination therapy (hydroxychloroquine sulfate-quinacrine hydrochloride or chloroquine phosphate-quinacrine), while 3 of them responded well to antimalarial monotherapy. The median time required to reach the response milestones on the final working therapeutic regimen was 3 months (mean, 4.8 months; range, 2-14 months). Six patients did not respond significantly to any type of therapy, including nonantimalarials. CONCLUSION: Our experience suggests that a significant subgroup of patients whose skin lesions have been unresponsive to a single antimalarial benefit from combination therapy with hydroxychloroquine and quinacrine or chloroquine and quinacrine, but controlled clinical trials are warranted to assess the extent of benefit.

Adult↗
PubMed2005

Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis.

BACKGROUND: Idiopathic inflammatory myopathies are chronic skeletal diseases with significant mortality and morbidity despite treatment by corticosteroids. Immunosuppressive agents and immunomodulatory therapy are used to improve disease control and reduce the long-term side effects of corticosteroids. While these treatments are used commonly in routine clinical practice, the optimal therapeutic regimen remains unclear. OBJECTIVES: To systematically review the evidence for the effectiveness of immunosuppressants and immunomodulatory treatments for dermatomyositis and polymyositis. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group trials register (searched February 2002 and updated in November 2003) and MEDLINE (January 1966 to December 2002). We checked bibliographies of identified trials and wrote to disease experts. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials including patients with probable or definite dermatomyositis and polymyositis as defined by the criteria of Bohan and Peter or definite, probable or mild/early by the criteria of Dalakas. Patients with inclusion body myositis should have been excluded by muscle biopsies. Any immunosuppressant or immunomodulatory treatment including corticosteroids, azathioprine, methotrexate, ciclosporin, chlorambucil, cyclophosphamide, intravenous immunoglobulin, interferon and plasma exchange was considered. Primary outcome was assessment of muscle strength after at least six months. Other outcomes were: change in disability, number of relapses and time to relapse, number of patients in remission and time-to-remission, cumulative corticosteroid dose and serious adverse effects. DATA COLLECTION AND ANALYSIS: Two authors (EC and JH) independently selected trials for inclusion in the review. Four authors independently assessed each study. Methodological criteria and the results of each study were recorded on data extraction forms. MAIN RESULTS: Seven potentially relevant randomised controlled trials were identified. One trial was excluded. Three studies compared immunosuppressant with placebo control, one trial compared one immunosuppressant (methotrexate) with another (azathioprine), another trial compared ciclosporin A with methotrexate and the final trial compared intramuscular methotrexate with oral methotrexate plus azathioprine. The study comparing intravenous immunoglobulin with placebo concluded that the former was superior. Two randomised placebo-controlled trials assessing plasma exchange, leukapheresis and azathioprine produced negative results. The fourth study compared azathioprine with methotrexate and found azathioprine and methotrexate equally effective but methotrexate had a better side effect profile. The fifth study comparing ciclosporin A with methotrexate and the sixth study comparing intramuscular methotrexate with oral methotrexate plus azathioprine found no statistically significant differences between the treatment groups. Immunosuppressants are associated with significant side effects. AUTHORS' CONCLUSIONS: This systematic review highlights the lack of high quality randomised controlled trials that assess the efficacy and toxicity of immunosuppressants in inflammatory myositis.

Azathioprine↗
PubMed1979

Dermatomyositis and toxoplasmosis.

In a patient with childhood dermatomyositis, high toxoplasma antibodies were found at the time of diagnosis. A direct immunofluorescence technique demonstrated active toxoplasmosis in the muscle biopsy. The response to treatment and follow-up in this patient suggest that toxoplasmosis could have caused the dermatomyositis.

Child↗
PubMed2003

US incidence of juvenile dermatomyositis, 1995-1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Registry.

OBJECTIVE: To estimate the incidence of juvenile dermatomyositis (juvenile DM) in the United States between 1995 and 1998. METHODS: Physician referrals to the National Institute of Arthritis and Musculoskeletal and Skin Diseases Juvenile Dermatomyositis Research Registry and the National Pediatric Rheumatology Disease Registry from Indiana University were utilized for a 2-source capture-recapture estimation of Juvenile DM annual incidence. RESULTS: For children 2-17 years of age, the estimated annual incidence rates from 1995 to 1998 in the US ranged from 2.5 to 4.1 juvenile DM cases per million children, and the 4-year average annual rate was 3.2 per million children (95% confidence interval 2.9-3.4). Estimated annual incidence rates by race were 3.4 for white non-Hispanics, 3.3 for African American non-Hispanics, and 2.7 for Hispanics. During the 4-year period of the study, completeness of ascertainment for the combined registries ranged from 56% to 86% and girls were affected more than boys (ratio 2.3:1). CONCLUSION: This study provides evidence for sex, and possibly racial differences in the risk of juvenile DM in the US.

Adolescent↗
PubMed1983

Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis.

Structural or functional abnormalities of the distal esophagus were demonstrated by cineradiography in 14 of 16 patients with polymyositis or dermatomyositis who had been incompletely responsive to high dose corticosteroids and, in some cases, immunosuppressive therapy, or who had adverse effects associated with these therapies. These distal abnormalities occurred in the absence of proximal esophageal skeletal muscle dysfunction in 70% of patients, usually were more frequent with increasing disease duration, and were functionally similar to abnormalities reported in scleroderma. Symptomatic improvement occurred to some patients with administration of antacids and measures designed to decrease esophageal reflux, but improved esophageal symptoms did not correlate with improvement in the myositis. In 18 autopsy subjects with similar histories, distal esophageal smooth muscle atrophy and fibrosis occurred in only 1 and 2 cases, respectively. Of 5 in whom distal esophageal dysmotility had been documented ante-mortem, only 1 had fibrosis and none had smooth muscle atrophy. These findings suggest that factors other than fibrosis and muscle atrophy may contribute to distal esophageal dysmotility in polymyositis and dermatomyositis.

Adult↗
PubMed1986

Prevalence of Coxsackie B virus antibodies in patients with juvenile dermatomyositis.

A number of viruses have been implicated as being the cause of various forms of myositis, including acute transient myositis, chronic polymyositis, and dermatomyositis. However, the cause of juvenile dermatomyositis (JDM) has remained elusive. Our study of serum samples taken within 4 months of the onset of disease in 12 children with JDM showed that 83% had detectable titers of complement-fixing (CF) antibody to 1 or more coxsackie B viral antigens. Detectable titers were found in only 25% of age-, sex-, and date-matched control sera taken from 24 patients with juvenile rheumatoid arthritis (JRA), and in 25% of serum samples taken from 2,192 "normal" children who had been hospitalized because of viral syndromes. Titers of CF antibody to coxsackie B1, B2, and B4 were positive in 58%, 50%, and 58%, respectively, of the JDM patients. In matched JRA controls, the respective values were 8%, 13%, and 8%. There were no significant antiviral titers and no significant differences in the results of tests for 13 other viral CF antigens, hepatitis B surface antigen, and Mycoplasma pneumoniae CF antigen in the JDM patient sera compared with the JRA patient sera. When titers of neutralizing antibody were determined, 58%, 58%, and 67% of the JDM patients were positive for coxsackie B2, B4, and B5, respectively, whereas 16%, 26%, and 21%, respectively, of the JRA controls were positive for the 3 antigens. These data suggest that the host response to coxsackie B virus might be related to the pathophysiology of JDM.

Antibodies, Viral↗
PubMed1993

Polymorphism of the cardiac manifestations in dermatomyositis.

Cardiac involvement in polymyositis and dermatomyositis usually is asymptomatic and rarely the principal clinical feature at the time of initial presentation. We describe a patient with dermatomyositis who presented with overt acute pericarditis which has not, to our knowledge, been previously reported. Acute pulmonary edema resulting from documented acute myocarditis developed during a second exacerbation. Long-term follow-up failed to demonstrate new cardiac complications during subsequent exacerbations, adding to the known polymorphism of the heart involvement in these systemic disorders.

Adult↗
PubMed2004

Dermatomyositis-like syndrome and HMG-CoA reductase inhibitor (statin) intake.

A patient developed an adult-onset dermatomyositis-like syndrome characterized by skin rash and progressive proximal muscle weakness concurrent with the intake of simvastatin. Despite discontinuation of the statin, symptoms progressed and required conventional steroid therapy for remission. The association between statins and the development of a musculocutaneous syndrome closely resembling dermatomyositis in susceptible subjects is poorly understood and has been reported rarely. The purpose of this report is to provide additional support for this pathological association. Since the population receiving statins is large and rapidly growing, caregivers are urged to be alert regarding the early recognition and proper care of the spectrum of neuromuscular complications linked to statin intake.

Aged↗
PubMed1988

Response to total body irradiation in dermatomyositis.

There were two patients with severe dermatomyositis refractory to immunosuppressive therapy treated with 150 rad of total body irradiation given over a period of 5 weeks. Both patients responded promptly with minimal side-effects and remain in partial remission 42 and 18 months after completion of the treatment. Total body irradiation is effective in some patients with dermatomyositis who are refractory to standard therapy.

Adult↗
PubMed1991

Magnetic resonance imaging of the muscles in patients with polymyositis and dermatomyositis.

Magnetic resonance imaging (MRI) of the muscles was performed in patients with polymyositis and dermatomyositis. Lesions with high intensity on T2-weighted image, but normal intensity on T1-weighted image, were observed in 7 of 8 patients in the active stage of the disease. Following clinical improvement with corticosteroid therapy in 4 patients, the high intensity lesions reverted to normal. The high intensity lesions seen on T2-weighted image in the active stage may represent edema and inflammation of the muscle. MRI of the muscle may serve as a diagnostic tool and be useful for follow-up of the patients with polymyositis or dermatomyositis.

Adult↗
PubMed1994

Tuberculous infection of skeletal muscle in a case of dermatomyositis.

A patient with dermatomyositis associated with carcinoma was treated with steroids and antibiotics for possible tuberculosis. Autopsy showed an overwhelming diffuse nongranulomatous infection of Mycobacterium tuberculosis involving only the skeletal muscles and one inguinal lymph node. The rare localization of tuberculosis to skeletal muscle in this case is possibly due to steroid immunosuppression and the humoral immune attack on muscle blood vessels that is a part of dermatomyositis.

Aged↗
PubMed1995

Disseminated histoplasmosis presenting as myositis and fasciitis in a patient with dermatomyositis.

A 54-year-old man with dermatomyositis initially responsive to corticosteroids and methotrexate developed severe myalgias, increasing weakness, and fevers. Laboratory studies were suggestive of disseminated histoplasmosis, and muscle biopsy revealed myositis, fasciitis, and yeast in the perimysial connective tissue. Histoplasma capsulatum was cultured from skeletal muscle. Despite antifungal therapy, necrotizing fasciitis progressed to gluteal abscess formation. Disseminated histoplasmosis may present atypically in immunocompromised hosts as fasciitis and myositis. Patients with dermatomyositis could be particularly vulnerable to soft tissue invasion by fungi due to their underlying microangiopathy.

Abscess↗
PubMed1989

Tubuloreticular structures (TRS) and cylindric confronting cisternae (CCC) in childhood dermatomyositis.

Tubuloreticular structures (TRS) and cylindric confronting cisternae (CCC) have been observed in circulating lymphocytes and in the muscle of six children with dermatomyositis. The presence of TRS was seen in all cases investigated, the number of CCC increased in various cells with the severity of the disease. Extensive formation of TRS and CCC in childhood dermatomyositis probably reflects local or systemic alpha-interferon production and suggests that some viral factor is responsible for the disease.

Child↗
PubMed1985

Familial occurrence of dermatomyositis and progressive scleroderma after injection of a local anaesthetic for dental treatment.

In an 11-year-old boy severe dermatomyositis occurred a few days after injection in the jaw of a local anaesthetic of the amide type for tooth extraction. The patient's grandmother was affected by progressive scleroderma at the age of 34 years, also after injection of a local anaesthetic for dental treatment. Both cases are discussed with special reference to the immuno-genetic aspects of the origin of dermatomyositis.

Adult↗