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PubMed1993

Correlation of serum neopterin concentrations with disease activity in juvenile dermatomyositis.

Serum neopterin concentrations were measured in 15 patients with juvenile dermatomyositis and their correlation with the severity of muscle impairment was evaluated. Neopterin concentrations were measured by radioimmunoassay. When compared with controls (median 0.75 ng/ml; range 0.33-1.18), serum neopterin concentrations were increased in patients with active juvenile dermatomyositis (median 3.21 ng/ml; range 0.95-13.81), but not in patients during remission (median 0.81 ng/ml; range 0.55-1.34). In horizontal and longitudinal studies, serum neopterin was significantly correlated with the severity of muscle strength impairment, whereas serum muscle enzyme values were not. These data suggest that measurement of serum neopterin may be used, together with clinical examination, to guide the treatment of patients with juvenile dermatomyositis.

Adolescent↗
PubMed1995

Intravenous immunoglobulin in juvenile dermatomyositis--four year review of nine cases.

Juvenile dermatomyositis is difficult to treat, compounded by complications of the disease itself as well as side effects of treatment. The mainstay of pharmacological management is corticosteroids, to which the disease is usually very responsive, but steroids have well established short and long term side effects. Refractory cases may be resistant to steroids or patients may become dependent on high doses, with relapse in clinical disease precipitated by reduction. Overtreatment with steroids and too rapid a reduction are common errors. Various second line agents have been used with success in complex or refractory cases, including cyclophosphamide, methotrexate, and cyclosporin. Intravenous immunoglobulin has been tried in the management of autoimmune diseases, either as an alternative to cytotoxic treatment or as a steroid sparing agent. The reported benefit in a number of childhood illnesses, including juvenile dermatomyositis, prompted this clinical trial in some of our more refractory cases. Over the past four years, nine children attending a dermatomyositis clinic have been treated with intravenous immunoglobulin. The two main indications were failure of conventional treatment and severe side effects from previous treatment, principally corticosteroids. All nine were still on treatment with various permutations of prednisolone, azathioprine, and cyclosporin. All nine showed clinical improvement at some point in their treatment with intravenous immunoglobulin. Of eight children on concurrent prednisolone, the dose could be reduced in six and kept the same in two.

Age of Onset↗
PubMed2006

Long-term outcome in polymyositis and dermatomyositis.

BACKGROUND: Although polymyositis and dermatomyositis are regarded as treatable disorders, prognosis is not well known, as in the literature long-term outcome and prognostic factors vary widely. AIM: To analyse the prognostic outcome factors in polymyositis and adult dermatomyositis. METHODS: We determined mortality, clinical outcome (muscle strength, disability, persistent use of drugs and quality of life) and disease course and analysed prognostic outcome factors. RESULTS: Disease-related death occurred in at least 10% of the patients, mainly because of associated cancer and pulmonary complications. Re-examination of 110 patients after a median follow-up of 5 years showed that 20% remained in remission and were off drugs, whereas 80% had a polycyclic or chronic continuous course. The cumulative risk of incident connective tissue disorder in patients with myositis was significantly increased. 65% of the patients had normal strength at follow-up, 34% had no or slight disability, and 16% had normal physical sickness impact profile scores. Muscle weakness was associated with higher age (odds ratio (OR) 3.6; 95% confidence interval (CI) 1.3 to 10.3). Disability was associated with male sex (OR 3.1; 95% CI 1.2 to 7.9). 41% of the patients with a favourable clinical outcome were still using drugs. Jo-1 antibodies predicted the persistent use of drugs (OR 4.4, 95% CI 1.3 to 15.0). CONCLUSIONS: Dermatomyositis and polymyositis are serious diseases with a disease-related mortality of at least 10%. In the long term, myositis has a major effect on perceived disability and quality of life, despite the regained muscle strength.

Adolescent↗
PubMed1986

Spontaneous pneumomediastinum in adult dermatomyositis.

Spontaneous pneumomediastinum has not been reported in adult polymyositis or dermatomyositis, either in conjunction with spontaneous pneumothorax or in isolation. Spontaneous pneumothorax has been rarely reported as a complication of rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and childhood dermatomyositis. It is associated with active, progressive pulmonary involvement and a poor prognosis. We describe an adult with dermatomyositis and spontaneous pneumomediastinum with a favourable outcome.

Adult↗
PubMed1990

Dermatomyositis following chronic staphylococcal joint sepsis.

A young man is reported with recurrent Staphylococcus aureus joint sepsis associated with dermatomyositis. His dermatomyositis failed to resolve on treatment with antimicrobial agents alone, indicating that if staphylococcal infection was the triggering event for the dermatomyositis then the subsequent process was apparently self perpetuating, requiring cytotoxic agents for its control. This case can be interpreted as possible further evidence for the triggering of autoimmune disease by infective agents.

Adolescent↗
PubMed1999

Interstitial lung disease in association with polymyositis-dermatomyositis: long-term follow-up CT evaluation in seven patients.

PURPOSE: To determine the long-term follow-up computed tomographic (CT) findings of interstitial lung disease associated with polymyositis-dermatomyositis. MATERIALS AND METHODS: CT scans in seven patients with interstitial lung disease and associated polymyositis-dermatomyositis were evaluated retrospectively. Six patients underwent sequential CT (follow-up range, 2-8 years; mean, 4.3 years). Histologic confirmation of pulmonary involvement was available in five patients. RESULTS: The predominant finding on the initial CT scans in four patients was subpleural consolidation, which corresponded to bronchiolitis obliterans organizing pneumonia with or without coexistent chronic eosinophilic pneumonia. In most cases, consolidation improved with use of corticosteroid and/or immunosuppressive therapy; in two patients, however, consolidation evolved into honeycombing. In one patient, diffuse areas of ground-glass opacity and consolidation appeared rapidly during illness; this patient died of sudden, rapid deterioration. In one patient with subpleural linear opacities, parenchymal abnormalities slowly progressed, and linear opacities had evolved into honeycombing at 8-year follow-up. In one patient with histologically proved organizing diffuse alveolar damage, bilateral patchy areas of ground-glass opacity and consolidation were seen. In one patient, subpleural bands changed to subpleural lines on sequential CT scans. CONCLUSION: CT provides an excellent demonstration of the lung changes in patients with interstitial lung disease and associated polymyositis-dermatomyositis.

Dermatomyositis↗
PubMed1989

Lymphocyte analysis in a patient with X-linked agammaglobulinemia and isolated growth hormone deficiency after development of echovirus dermatomyositis and meningoencephalitis.

A previously described patient with X-linked agammaglobulinemia and growth hormone deficiency developed an echovirus-associated meningoencephalitis and dermatomyositis-like syndrome while being treated with intramuscular gamma globulin and human growth hormone. Initiation of high-dose intravenous gamma globulin resulted in resolution of the clinical symptoms and the patient has remained asymptomatic over the past 55 months. Lymphocyte phenotype analysis at the time of presentation with echovirus infection revealed an increase in CD2+, CD16+, HNK-1+ lymphocytes, a decrease in CD4+ T cells as well as absence of B cells. This elevation in the LGL/NK phenotype resolved with clinical improvement. In addition, there was evidence of lymphocyte activation following the development of echovirus infection (increase in HLA-DR expression and elevated serum IL-2 receptor levels) which resolved with clinical improvement. A muscle biopsy obtained during the period of the dermatomyositis-like syndrome demonstrated a CD8+ lymphocytic infiltrate very similar to the observations in classical dermatomyositis. Taken together, these findings suggest that growth hormone therapy in this patient failed to alter the humoral immunodeficiency. In addition, serum IL-2 receptor levels and lymphocyte phenotyping may be useful adjuncts for monitoring echovirus disease in immunodeficient patients.

Agammaglobulinemia↗
PubMed1996

Low-dose methotrexate as an adjunctive therapy with surgery for ectropion complicating dermatomyositis.

We describe the clinical and histopathologic features of a 44-year-old woman who developed bilateral lower eyelid ectropion and upper lid entropion presumably secondary to dermatomyositis. A variety of inflammatory, infiltrative and/or scarring dermatoses have been associated with ectropion. Chronic inflammation, poikilodermatous change and mucinous infiltration of the eyelids due to dermatomyositis are the postulated mechanisms leading to ectropion in our patient. This case is unique in that neither dermatomyositis nor papular mucinosis has previously been reported as a cause of ectropion. Also interesting was the adjunctive effect of methotrexate therapy.

Adult↗
PubMed1985

Dermatomyositis associated with chronic myelogenous leukemia.

Dermatomyositis and polymyositis have been well established in association with malignant neoplastic disease. Most commonly, this association has been noted in patients with solid tumors rather than in patients with neoplasms of hematopoietic origin. We describe here a patient with chronic myelogenous leukemia who developed typical dermatomyositis, which responded to therapy with corticosteroids. This concurrence has been reported in only one previous patient to our knowledge. The nature and implications of the association of dermatomyositis and neoplasia are discussed.

Adult↗
PubMedDate not supplied

Spontaneous perforation of the esophagus as a manifestation of dermatomyositis.

Spontaneous perforation of the esophagus evolving into a retropharyngeal abscess in a child with severe dermatomyositis is reported. Clinical presentation and successful management are discussed. Aggressive surgical debridement with extensive drainage, multiple antibiotic coverage, and careful steroid management are advocated. The histopathology and clinical presentation of dermatomyositis as a type of polymyositis are discussed as pertinent to the otolaryngologic clinician. The literature is reviewed and results of a retrospective analysis of head and neck manifestations are reported in 50 children with dermatomyositis treated at Children's Hospital, Los Angeles, over the past 20 years.

Abscess↗
PubMed1993

Proton magnetic resonance imaging and spectroscopic studies of the pathogenesis and treatment of juvenile dermatomyositis.

Juvenile dermatomyositis is an inflammatory disorder of muscle, skin, and connective tissue. Immune vasculopathy is central to the pathophysiology. We studied a 13-year-old boy with juvenile dermatomyositis using proton magnetic resonance imaging (MRI) with short tau inversion recovery (STIR), and proton magnetic resonance spectroscopy (MRS) to quantitate lipid and water in affected regions of muscle. Tissue perfusion was assessed by measuring tissue water concentration changes during isometric exercise of the tibialis anterior muscle. During sequential studies over 3 months of steroid treatment, STIR image abnormalities, resting water concentrations, and diminished perfusion returned to normal. Resting lipid concentrations increased during this period. MRI serves to guide muscle biopsy and monitor progress of the disease state. MRS demonstrates the vasculopathy and provides noninvasive assessment of steroid therapy in juvenile dermatomyositis.

Adolescent↗
PubMed2003

Primary non-Hodgkin's lymphoma of the transverse colon presenting as dermatomyositis: case presentation and literature review.

Primary extranodal non-Hodgkin's lymphoma of the transverse colon is a rare presentation of non-Hodgkin's lymphoma or colonic neoplasm. Dermatomyositis is an autoimmune condition of the skin, muscle, and blood vessels that when associated with malignancy is a true paraneoplastic syndrome but is rarely associated with non-Hodgkin's lymphoma. We present a case of primary non-Hodgkin's lymphoma of the transverse colon diagnosed after the presentation of dermatomyositis and review the literature on dermatomyositis and hematologic neoplasm.

Aged↗
PubMed2006

Management of cutaneous dermatomyositis: current therapeutic options.

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations and proximal muscle weakness. Patients with this condition frequently require treatment for muscular, systemic, and cutaneous involvement; however, the cutaneous symptoms often cause significant morbidity and frustration to patients. The skin lesions of dermatomyositis often persist or recur after treatment of myositis, and there are subsets of patients who have significant cutaneous disease with little or no muscle involvement. Only anecdotal evidence is available for cutaneous treatment, and the approach to therapy is guided by disease severity and refractoriness. First-line therapy for localized disease begins with topical agents such as antipruritics and/or corticosteroids, while widespread disease often requires initial systemic therapy. Antimalarials are generally used for significant skin disease, followed by other anti-inflammatory agents, systemic corticosteroids, corticosteroid-sparing immunosuppressants and, recently, biologics and intravenous immunoglobulin. While there is a recognized absence of randomized prospective clinical trials examining the optimal monotherapy and combination treatment for the cutaneous manifestations of dermatomyositis, we have tried to provide a review of the literature and a systematic approach to dealing with these often refractory and debilitating symptoms.

Administration, Topical↗
PubMed1997

[An adult case of dermatomyositis complicated with cecum perforation and panniculitis].

The case of dermatomyositis complicated with cecum perforation and panniculitis occurred in a 62-year-old woman was reported. She was admitted to Keio University Hospital with a history of proximal muscular weakness, and dysphagia. Physical examination showed erythema over the face and shoulder. Serum level of muscle enzymes was remarkably increased. The diagnosis of dermatomyositis was made based on proximal muscular weakness, elevated serum level of muscle enzymes and myogenic change of electromyocardiogram. The treatment with 60 mg/day of prednisolone was started, and was a good response. However, 7 months later the disease became active again when the amount of prednisolone was reduced to 13 mg/day. Subsequently she complained of abdominal pain on the right lower quadrant. The surgical findings included peritonitis due to the perforation of the cecum and multiple ulcers of the cecum. After operation, azathioprine was added. Four years and 9 months later, she noticed skin erythema with ulceration and subcutaneous nodule. Skin biopsy indicated the findings of the panniculitis with membrano-cystic lesion. It was thought that both cecum perforation and panniculitis were caused by angiopathy which was often seen in childhood dermatomyositis.

Cecal Diseases↗
PubMed2005

[Systemic lupus erythematosus and dermatomyositis--case report].

The association of systemic lupus erythematosus (SLE) with idiopathic polymyositis or dermatomyositis is reported to occur in the range of 4-16%. Myositis can occur before or after SLE, or sporadically both diseases can be present simultaneously. This case report concerns a 36-year-old female patient suffering from Raynaud's phenomenon, polyarthralgia in the small joints of the hands, and skin changes compatible with Gotron's indications. Symmetric proximal muscle weakness of the extremities, fever of up to 40 degrees C, heliotrope rashes with erythematous changes in the face, upper arms, and posterior shoulders occurred subsequently. Laboratory analyses revealed increased acute phase reactants, hypochromic anaemia, lymphopenia, and increased levels of all muscle enzymes. Immunoserology demonstrated positive ANA, anti-Sm, and anticardiolipin antibodies (aCL), while anti dsDNA, anti Ro, anti La, and anti Jo-1 antibodies proved negative. Hypocomplementaemia and elevated levels of immune complexes were also detected. Pathologic sediment and proteinuria were revealed via urine analyses, while a kidney biopsy confirmed lupus nephritis (type IVa according to the World Health Organisation classification). Biopsy of erythematous changes of the posterior shoulder demonstrated leukocytoclastic vasculitis. Electromyography of the lower extremities established myopathic changes. Inflammation of the muscles was confirmed via magnetic resonance imaging. The patient was categorised as having two separate coexistent diseases--SLE and dermatomyositis. Both the classification criteria of the American College of Rheumatology for SLE and the diagnostic criteria for dermatomyositis, proposed by Bohon and Peter, were fulfilled simultaneously. Treatment commenced with pulses of methylprednisolone and continued with oral therapy, including Resochin. Pulses of intravenous cyclophosphamide were also administered. After six weeks of therapy, biohumoral remission of both diseases was achieved, while complete recovery from muscle weakness was accomplished after four months.

Adult↗
PubMed2006

Cytomegalovirus infection complicating immunosuppressive therapy for dermatomyositis.

The occurrence of opportunistic infections is not rare during the treatment of connective tissue diseases, including dermatomyositis because of the patient's immunosuppression. We describe here cytomegalovirus (CMV) infection that affected two of our patients with dermatomyositis. CMV infection has not been considered to be a frequent event in dermatomyositis, and its significance for disease progression has not been sufficiently recognized. Our case reports demonstrate the importance of close monitoring of such patients and prompt initiation of appropriate therapy to manage a suspected CMV infection.

Aged↗
PubMed1996

Cell adhesion molecule expression in cutaneous lesions of dermatomyositis.

Chronic inflammation seems to play a major role in skin and muscle cell damage in dermatomyositis. Adhesion molecules and their ligands are fundamental in regulating inflammation. We have carried out an immunohistochemical analysis of different activation-inducible adhesion markers in 15 biopsy specimens from dermatomyositis skin lesions. Consistent findings were the increased expression of intercellular adhesion molecule-1 (ICAM-1) on endothelial cells, inflammatory cells and focally grouped keratinocytes in contact with subepidermal inflammatory infiltrates. Immunoreactivity for vascular cell adhesion molecule-1 (VCAM-1) was predominant on endothelial cells of the upper reticular dermis and dermal stellate-shaped cells. E-selectin (endothelial leukocyte adhesion molecule-1) immunoreactivity was less extensive, detected mostly on segments of vessels of the papillary dermis and upper reticular dermis, and sometimes independent of inflammation. This pattern of adhesion molecule expression is similar to that described in other immunemediated dermatoses. The up-regulation of the adhesion molecules appears to play a role in the development and perpetuation of dermatomyositis skin lesions.

Adolescent↗
PubMed2003

Dermatomyositis developing in the first trimester of pregnancy.

Dermatomyositis in pregnancy is rare. Pregnancy may be a precipitating factor at the onset or may develop during the course of dermatomyositis, which would exacerbate disease activity. In this study, we report a 22-year-old female patient who developed generalized skin rash and progressive muscle weakness in the twelfth week of pregnancy. She was diagnosed with dermatomyositis and underwent therapeutic abortion, due to the high fetal mortality rate of the disease when developed in the first trimester. Her symptoms improved with treatment of intravenous immunoglobulin and a high dose of corticosteroids.

Adult↗