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PubMed2003

Dermatomyositis associated with angiotropic lymphoma.

We describe a 64-year-old man with dermatomyositis sine myositis who presented with unusual cutaneous plaques and nodules. Initial investigations failed to reveal evidence of malignancy. Eighteen months later he became systemically unwell and repeat biopsies from the nodules confirmed angiotropic T-cell lymphoma. Cutaneous lymphoma is rarely reported with dermatomyositis and in the previously reported cases, mycosis fungoides was the variant seen. This is the first report of angiotropic T-cell lymphoma associated with dermatomyositis.

Antineoplastic Agents, Hormonal↗
PubMedDate not supplied

Periorbital edema as the presenting sign of juvenile dermatomyositis.

We report a case of juvenile dermatomyositis that presented with periorbital edema. Dermatomyositis is an autoimmune disorder with cutaneous manifestations including heliotrope patches, Gottron's papules, periungual telangiectasisas, and subcutaneous calcifications. Periorbital edema may accompany the classic heliotrope rash and, as in this case, may be the only presenting sign of juvenile dermatomyositis.

Child, Preschool↗
PubMed1992

Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis.

BACKGROUND: The therapeutic options for patients with polymyositis or dermatomyositis that is resistant to corticosteroids are limited, unproved, and often toxic. Uncontrolled trials concluded that both plasma exchange and leukapheresis are beneficial, but despite the considerable use of these approaches, proof of their efficacy is lacking. METHODS: Thirty-nine patients with definite polymyositis or dermatomyositis were randomly assigned to receive plasma exchange (replacement of one volume of plasma with 5 percent albumin in saline), leukapheresis (removal of 5 x 10(9) to 10 x 10(9) lymphocytes), or sham apheresis in a double-blind manner, with 12 treatments given over a one-month period. Muscle strength, functional capacity, and serum levels of muscle-associated enzymes were measured before and after the 12 procedures. RESULTS: In each group 3 of 13 patients had improvements in strength and functional capacity. The condition of 3 patients treated with leukapheresis and 1 treated with plasma exchange deteriorated, and it was unchanged in the other 26 patients. Adverse effects of apheresis included the need for a central venous catheter (9 patients), major vasovagal episodes (3 patients), and severe citrate reactions (2 patients). Despite the occurrence of significant reductions in the serum levels of muscle enzymes with plasma exchange (P less than 0.001) and significant decreases in lymphocyte counts with leukapheresis (P = 0.002), there were no significant differences among the three treatment groups in the final muscle strength or functional capacity of the patients. CONCLUSIONS: As treatments for corticosteroid-resistant polymyositis or dermatomyositis, leukapheresis and plasma exchange are no more effective than sham apheresis.

Activities of Daily Living↗
PubMed1993

A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis.

BACKGROUND: Dermatomyositis is a clinically distinct myopathy characterized by rash and a complement-mediated microangiopathy that results in the destruction of muscle fibers. In some patients the condition becomes resistant to therapy and causes severe physical disabilities. METHODS: We conducted a double-blind, placebo-controlled study of 15 patients (age, 18 to 55 years) with biopsy-proved, treatment-resistant dermatomyositis. The patients continued to receive prednisone (mean daily dose, 25 mg) and were randomly assigned to receive one infusion of immune globulin (2 g per kilogram of body weight) or placebo per month for three months, with the option of crossing over to the alternative therapy for three more months. Clinical response was gauged by assessing muscle strength, neuromuscular symptoms, and changes in the rash. Changes in immune-mediated muscle abnormalities were determined by repeated muscle biopsies. RESULTS: The eight patients assigned to immune globulin had a significant improvement in sores of muscle strength (P < 0.018) and neuromuscular symptoms (P < 0.035), whereas the seven patients assigned to placebo did not. With crossovers a total of 12 patients received immune globulin. Of these, nine with severe disabilities had a major improvement to nearly normal function. Their mean muscle-strength scores increased from 74.5 to 84.7, and their neuromuscular symptoms improved. Two of the other three patients had mild improvement, and one had no change in his condition. Of 11 placebo-treated patients, none had a major improvement, 3 had mild improvement, 3 had no change in their condition, and 5 had worsening of their condition. Repeated biopsies in five patients of muscles whose strength improved to almost normal showed an increase in muscle-fiber diameter (P < 0.04), an increase in the number and a decrease in the diameter of capillaries (P < 0.01), resolution of complement deposits on capillaries, and a reduction in the expression of intercellular adhesion molecule 1 and major-histocompatibility-complex class I antigens. CONCLUSIONS: High-dose intravenous immune globulin is a safe and effective treatment for refractory dermatomyositis.

Adolescent↗
PubMed2001

Juvenile dermatomyositis: a retrospective review of a 30-year experience.

BACKGROUND: Children with juvenile dermatomyositis (JDMS) have variable initial presentations. OBJECTIVE: Our purpose was to evaluate the epidemiology trends, presenting clinical features, laboratory data, and outcome of patients with JDMS. METHODS: A total of 16 patients were identified at Geisinger Medical Center by a 30-year retrospective chart review. RESULTS: Sex ratio, age at diagnosis, and outcome were similar to data published in previous studies. However, certain trends were noted. The most common initial physical examination findings were an extremity rash (94%) and periungual erythema (75%). New associations of JDMS that were uncovered included the findings of pruritus (38%) and a psoriasiform scalp dermatitis (25%). Nonspecific laboratory elevations were the most common initial laboratory changes (erythrocyte sedimentation rate, lactate dehydrogenase, and aspartate aminotransferase). Tubuloreticular inclusions as found on electron microscopy of muscle biopsy specimens were present in all 3 patients tested. One patient with tubuloreticular inclusions had otherwise normal muscle biopsy findings on hematoxylin-and-eosin staining. Two of the 16 patients had cutaneous findings of JDMS but did not exhibit muscle involvement after long-term follow-up at 4 and 5 years. CONCLUSION: Our study confirms that the initial physical and laboratory findings in patients with JDMS may be nonspecific. The heliotrope rash and Gottron papules classically associated with dermatomyositis appeared less commonly than an extremity rash and periungual erythema. Creatinine kinase and aldolase levels may not be elevated on initial presentation. Pruritus, a psoriasiform scalp dermatitis, and tubuloreticular inclusions found on muscle biopsy electron microscopy should be additional factors to consider. The long-term follow-up in 2 patients without muscle involvement lends support to the existence of amyopathic dermatomyositis.

Adolescent↗
PubMed2001

Juvenile dermatomyositis presenting with anasarca: A possible indicator of severe disease activity.

Juvenile dermatomyositis is a rare autoimmune disease characterized by inflammation of the muscle, skin, and other organs. Although localized edema is a common feature of juvenile dermatomyositis, generalized edema has been reported infrequently. We describe a patient with juvenile dermatomyositis presenting with anasarca and note that generalized edema has been associated with severe disease activity.

Child↗
PubMed2002

Dermatomyositis in 132 patients with different clinical subtypes: cutaneous signs, constitutional symptoms and circulating antibodies.

We retrospectively studied 132 patients with dermatomyositis; 84 had idiopathic, 30 paraneoplastic, 5 juvenile and 13 amyopathic forms of the disease. The commonest features were macular erythema, heliotropic erythema and Gottron's papules. Flagellate erythema occurred in 5% of patients with idiopathic dermatomyositis and correlated with the disease activity. Necrotic lesions were also found in this group of patients but did not always signal malignancy. The prevalence of malignancy was high (23%). Raynaud's phenomenon occurred in 10.6% of patients, also in those with malignancy. Dysphagia, interstitial lung disease and arthralgias affected 20%, 8% and 40% of patients, respectively. Anti-Jo-1 antibodies were found in 5% of patients with idiopathic dermatomyositis and low titre ANA in 1/3 of patients. ANA did not correlate with the disease activity. We confirmed the data from the literature, but no cutaneous sign, constitutional symptom or circulating antibody was found marking a particular subtype of the disease.

Adolescent↗
PubMed1995

Juvenile dermatomyositis in a child infected with human parvovirus B19.

We report a case of juvenile dermatomyositis in which clinical onset occurred soon after infection with human parvovirus B19. To the best of our knowledge an association between juvenile dermatomyositis and human parvovirus B19 has not been previously reported. Parvovirus infection has been associated with several autoimmune diseases, and the findings in the case we describe raise the possibility that parvovirus may induce juvenile dermatomyositis, although we could not demonstrate a causal relationship.

Anti-Inflammatory Agents↗
PubMed1996

Use of sequential DTPA clearance and high resolution computerized tomography in monitoring interstitial lung disease in dermatomyositis.

Interstitial lung disease complicating polymyositis-dermatomyositis has a grave prognosis. We report the case of a 50-yr-old woman with dermatomyositis and interstitial lung disease monitored by sequential high-resolution computerized tomography (HRCT) of lung and aerosol clearance times of the radionuclide 99Technetium-diethylene triamine pentacetate (DTPA). She was treated with oral cyclophosphamide and prednisolone with good outcome. Pulmonary response to therapy was followed with sequential HRCT and DTPA scanning. DTPA clearance a measure of lung inflammation, and HRCT paralleled clinical course during the treatment of interstitial lung disease. Sequential HRCT and DTPA were useful adjuncts in the initial assessment and monitoring of interstitial lung disease in association with dermatomyositis.

Anti-Inflammatory Agents↗
PubMed1988

Immunoperoxidase technique on paraffin sections fails to demonstrate immunoglobulin deposition in cutaneous lesions from patients with dermatomyositis.

Nine formalin-fixed paraffin sections of skin biopsy specimens of patients with clinically and histologically proven dermatomyositis were studied using the avidin-biotin immunoperoxidase technique. No evidence of dermoepidermal junction or perivascular IgG, IgA, or IgM deposits were noted. These findings may be related to loss of antigenicity during the fixation process or to the small amount of immunoglobulin deposit previously reported in dermatomyositis. The negative findings demonstrate an inability to use this technique to find immunoglobulin deposition retrospectively in dermatomyositis.

Adolescent↗
PubMed1997

Dermatomyositis and malignant melanoma.

Patients with dermatomyositis may face an increased risk of malignancy. Malignant melanoma has been linked to dermatomyositis. We present a case of concomitant dermatoyositis and metastatic malignant melanoma and review the English literature concerning this topic. Analysis revealed that the development of dermatomyositis in patients with malignant melanoma heralds the diagnosis of metastatic disease. These patients face dismal prognoses.

Adult↗
PubMed1995

An update on juvenile dermatomyositis.

Juvenile dermatomyositis (JDMS) is a systemic vasculopathy characterized primarily by inflammation of skin and muscle. JDMS is identified in more than three per million persons per year, using established diagnostic criteria. Although originally thought to be a relatively homogeneous disease, new data confirm that heterogeneity in JDMS may be found at several levels and that each variant may be associated with a different disease course. Unlike adults with dermatomyositis, of whom more than 50% have a specific myositis-associated antibody (MSA), a much smaller number of children appear to test positive for a known MSA (about 10%), despite the evidence that more than 60% of children with JDMS test positive for antinuclear antibodies. In children, the most common MSA is directed against Mi-2, not toward one of the tRNA synthetases, such as tRNA histidine, as is found in 20% to 30% of adults with myositis. About 50% of children with JDMS have circulating evidence of endothelial cell damage (increased vWF:Ag), whereas others have different indicators of disease activity, such as elevated neopterin (> 60%) or increased circulating B cells with peripheral lymphopenia (> 80%). Newer modes of assessment of functional ability may help evaluate response to therapy. Finally, physicians with newly diagnosed (< 6 months) JDMS patients are urged to call the new National Institutes of Health Rare Disease Registry for New Onset Dermatomyositis (312-880-3333) to enroll their patients and for more information on the onset of this disease.

Anti-Inflammatory Agents↗
PubMed1993

Amyopathic dermatomyositis: a review.

Jim Gilliam's research interests throughout his career were forced upon better defining the relationships that exist between the cutaneous and systemic manifestations of the rheumatic diseases. Although the majority of his time was spent studying such relationships in lupus erythematosus patients, he was also intensely interested in dermatomyositis (DM) in this regard as well. He was particularly intrigued with the dissociation of the cutaneous and muscular manifestations of this disorder that occasionally occurs. The term "dermatomyositis siné myositis" has been used in the past to describe patients who present with only the cutaneous manifestations of DM; however, very little published data is available from systematic examinations of such patients. For several reasons, we have preferred the term "amyopathic dermatomyositis" to describe that rare patient who for long periods of time suffers from the classical skin lesions of DM as the only clinically significant manifestation of their disease. In this presentation, we review our own personal experience with a group of six such patients and compare and contrast it to that of other workers who have dealt with this subject over the past two decades.

Dermatomyositis↗
PubMed1997

Dermatomyositis associated with primary intramuscular B cell lymphoma.

A rare case of dermatomyositis associated with primary intramuscular malignant lymphoma is described. A 40-year-old Japanese woman noticed swelling of the right thigh during the treatment of dermatomyositis with prednisolone, azathioprine and cyclophosphamide. A biopsy specimen taken from the right quadriceps muscle revealed infiltration of lymphoma cells which were positive for CD20. We reviewed 12 cases of dermatomyositis associated with malignant lymphoma reported in Japanese literature between 1984 and 1996.

Adult↗
PubMed1999

Subcutaneous emphysema with spontaneous pneumomediastinum and pneumothorax in adult dermatomyositis.

We describe a 32-year-old patient with adult dermatomyositis who developed dyspnea and worsening of pre-existing infarcted skin lesions of the fingers. Chest radiographs showed diffuse hazy reticulonodular infiltration in both lungs, subcutaneous emphysema, pneumomediastinum, and pneumothorax. The pulmonary symptoms and cutaneous lesions gradually improved with a high dose of prednisolone. Although subcutaneous emphysema and pneumomediastinum occur frequently in association with traumatic disruption of cutaneous and mucosal barriers and assisted ventilation, it has rarely been observed in patients with interstitial pneumonitis in connective tissue diseases. Although dermatomyositis and subcutaneous emphysema are all relatively well-known diseases to dermatologists, the occurrence of spontaneous pneumomediastinum and pneumothorax and subsequent subcutaneous emphysema in connective tissue diseases such as dermatomyositis is unfamiliar. We discuss the possible mechanisms of this condition.

Adult↗
PubMed1994

Cutaneous photosensitivity in dermatomyositis.

The incidence and nature of cutaneous photosensitivity were studied in 10 patients suffering from dermatomyositis. Five reported an abnormality, which consisted of photoaggravation of preexisting cutaneous lesions in three, and abnormal transient erythemal responses in two. Monochromatic irradiation testing of all 10 patients demonstrated reduced minimal erythemal doses in two, at 307.5 nm, and at 340 and 360 nm, respectively; only the latter individual had clinical light sensitivity. Exposure to low-dose, solar-simulated radiation of the unaffected skin of the former patient, and five others who agreed to the procedure, three of whom complained of light sensitivity, induced a lesion with the clinical and immunofluorescence characteristics of dermatomyositis in only the first one. Four other patients replied to a mailed questionnaire, and three of these reported aggravation of their rash and provocation of new lesions by sunlight. Photosensitivity may thus be an important cutaneous feature of dermatomyositis.

Adult↗
PubMed2005

Advanced renal pelvic carcinoma associated with dermatomyositis.

Dermatomyositis is an uncommon in flammatory myopathy with characteristic cutaneous manifestations which is frequently linked to several cancers. A 42-year-old man presented with left flank pain with typical symptoms of dermatomyositis. Computed tomography showed a solid mass in the pelvis of the left kidney and lymphadenopathy in the retroperitoneum. Since the general condition of the patient rapidly deteriorated because of marked muscle weakness of the respiratory muscles, the patient initially underwent medical treatment with prednisolone. After the improvement of respiratory function, the patient underwent radical nephroureterectomy and retroperitoneal lymphadenectomy. The pathological specimen showed moderately differentiated adenocarcinoma, stage pT3N2. After surgery, the patient showed a marked improvement of clinical symptoms related to dermatomyositis. Twenty-two months later, multiple organ metastases occurred and the patient died of cancer. We should point out the significance of surgical and medical treatment for these patients.

Adenocarcinoma↗
PubMedDate not supplied

Dermatomyositis following the diagnosis of ovarian cancer.

We present a case history of a woman who developed dermatomyositis following the diagnosis of stage IV ovarian cancer. Dermatomyositis is a rare paraneoplastic syndrome that usually precedes the diagnosis of ovarian cancer by several months or years. Ours is the fifth reported case of dermatomyositis after an established diagnosis of ovarian cancer in the literature.

Aged↗