A patient with cutaneous T-cell lymphoma and dermatomyositis.
Dermatomyositis in adults has been associated with a variety of internal malignancies. We present a case of a patient with dermatomyositis and cutaneous T-cell lymphoma.
Aged↗
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[Primary pulmonary non-Hodgkin's lymphoma in a patient with dermatomyositis].
A 47-year-old man with dermatomyositis and interstitial pneumonia had been treated with prednisolone since May, 1992, and with azathioprine since April, 1993. During the sixth month of this treatment, primary pulmonary non-Hodgkin's lymphoma (T-cell, diffuse, pleomorphic) developed. Chemotherapy (vincristine and adriamycin) was begun but there was no response. An invasive lesion of the brain was seen on a CT image. Despite cranial radiotherapy, the patient died of respiratory suppression due to progressive brain disease on December 14, 1993. Primary pulmonary non-Hodgkin's lymphoma develops only rarely in patients with dermatomyositis. In this case, oncogenesis may have been related to the use of immunosuppressants.
Azathioprine↗
[A case of amyopathic dermatomyositis associated with interstitial pneumonitis].
A patient (47-year old female) who had erythema similar to Gottron's sign on bilateral finger joints since two years ago, started to have polyarthralgia on bilateral knee and shoulder on the spring of 1993. Polyarthralgia was extended to both wrist and hand joints on Oct. of 1995. On the middle of Dec. 1995, she began to have exertional dyspnea and was referred and admitted in our hospital on 18th, Dec., 1995. Chest X-ray and CT scan showed the shadow for active interstitial pneumonitis on bilateral lower lung fields. Blood gas analysis indicated hypoxia (PaO2: 62.8 mmHg) and low % DLCO (64.7%). Skin eruption of face (heliotrope-like erythema) and hands (Gottron's sign) and skin biopsy (right hand) findings were compatible with that in dermatomyositis. The analysis in blood biochemistry showed no elevation for muscle enzymes. The diagnosis for amyopathic dermatomyositis (ADM) was made according to the criteria proposed by Euwer & Sontheimer (1993). The steroid pulse therapy and 50 mg per day of cyclophosphamide were immediately administered. The dyspnea and dermatitis were improved within two weeks after therapy. She is presently in remission until Jan. 1997 with the maintenance dosis of 10 mg per day of oral prednisolone.
Anti-Inflammatory Agents↗
[Internuclear bilateral pseudo-ophthalmoplegia and dermatomyositis].
A 60 year-old woman complaining of diplopia presents an ocular motility disturbance mimicking internuclear ophthalmoplegia. Idiopathic dermatomyositis is diagnosed by the help of clinical, biological, electrophysiological and histological data. The outcome is favorable under corticotherapy. Ocular muscle involvement is rare in dermatomyositis. An overlap syndrome with another auto-immune disorder like myasthenia should be excluded in this kind of manifestation.
Dermatomyositis↗
Dermatomyositis induced by penicillamine.
A case of dermatomyositis is reported in a 50-year-old woman receiving D-Penicillamine therapy for rheumatoid arthritis. There was no evidence of neoplasia on full investigation. Remission of dermatomyositis occurred on withdrawal of D-Penicillamine.
Arthritis, Rheumatoid↗
Diminished class II-associated Ii peptide binding to the juvenile dermatomyositis HLA-DQ alpha 1*0501/DQ beta 1*0301 molecule.
HLA class II molecules bind and present peptide Ags to T cells, binding specific sets of peptides due to polymorphism in the peptide binding groove. Class II proteins associate with the invariant chain (Ii chain) and its derived class II-associated Ii peptide (CLIP). Ii chain association is important for normal trafficking of class II proteins to the peptide loading vesicles and for blocking premature access of peptides to HLA class II molecules during maturation. We have previously shown that juvenile dermatomyositis is associated with the HLA-DQA1*0501 allele. There is limited information available about the interaction of any DQ molecule with the Ii chain and little information about binding of individual peptides to HLA-DQalpha1*0501/DQbeta1*0301. We sequenced peptides eluted from the juvenile dermatomyositis-associated class II allele HLA-DQalpha1*0501/DQbeta1*0301. Surprisingly, we found no Ii chain or CLIP. Further examination of peptide binding to the HLA-DQalpha1*0501/DQbeta1*0301 molecule demonstrated poor CLIP binding. However, newly synthesized HLA-DQalpha1*0501/DQbeta1*0301 molecules do associate with intact Ii chain. Molecular modeling suggests that CLIP binds differently to HLA-DQalpha1*0501/DQbeta1*0301 than to DR molecules. The lack of CLIP association suggests that HLA-DQalpha1*0501/DQbeta1*0301 has access to peptides earlier in the processing pathway and so might encounter novel peptides that induce autoimmunity.
Alleles↗
Cutaneous ulcers of refractory adult dermatomyositis responsive to intravenous immunoglobulin.
We report the case of a woman from the Bahamas who presented with many of the classic manifestations of dermatomyositis. She responded well to initial therapy, which included oral corticosteroids, low-dosage methotrexate, and hydroxychloroquine. The cutaneous component of her disease flared dramatically months later, and progressed despite aggressive therapy with the higher dosages of the same medications. Therapy with intravenous immunoglobulin (IVIG) was initiated and the patient stopped forming new cutaneous ulcers within 1 to 2 weeks. Theories about the mechanism of action of IVIG, and practical guidelines for its use in treating patients with dermatomyositis, are briefly reviewed.
Dermatologic Agents↗
Adult dermatomyositis with livedo reticularis and multiple skin ulcers.
Skin ulcers are commonly described in the juvenile form of dermatomyositis; however, in the adult form they are rarely described. We report on a patient with adult dermatomyositis, deep cutaneous ulcers in the skin folds and livedo reticularis eruption.
Abdomen↗
Dermatomyositis following rhabdomyolysis.
A case is presented, in which classic dermatomyositis occurred six weeks after documented rhabdomyolysis. The literature concerning the immunopathogenesis of dermatomyositis is briefly reviewed.
Adult↗
Dermatomyositis with subclinical myositis and spontaneous pneumomediastinum with pneumothorax: case report and review of the literature.
Dermatomyositis (DM) is a clinical entity characterized by a distinctive cutaneous rash and inflammatory myopathy. Besides skin and muscle, the disease can also involve other internal organs, especially the lungs. We describe a patient with dermatomyositis and incomplete signs of myositis who developed pneumomediastinum, pneumothorax and massive subcutaneous emphysema. This case illustrates a rare pulmonary complication of DM, and underscores that muscular involvement in this disease is not always reflected in laboratory and/or histological abnormalities.
Adult↗
[Recurrent pneumomediastinum in the course of interstitial pneumonia associated with dermatomyositis].
A 59-year-old woman with dermatomyositis suffered pneumomediastinum twice during steroid therapy for aggravated myositis. In both instances, pneumomediastinum occurred when the patient's aggravated myositis was in remission. Plain chest X-ray films showed a slit-like air lucency around the left pulmonary artery. This indicated that air entered the mediastinum through the left hilum. Although pneumomediastinum associated with dermatomyositis is considered to be fatal, in our patient each instance of pneumomediastinum spontaneously disappeared very quickly. The favorable course of our patient seemed to be associated with her high elevated levels of serum creatine kinase and seropositive results for anti-Jo-1 antibody.
Dermatomyositis↗
Recurrent Breast Cancer at 21 years after Resection Detected by Serum Tumor Markers and Manifested as Dermatomyositis.
A 52-year-old Japanese woman developed dermatomyositis. She had undergone a standard radical mastectomy for left breast cancer 21 years earlier. Though no physical sign of recurrent breast cancer appeared clinically, levels of tumor markers were abnormally elevated. Therefore, tamoxifen and CAF therapy were given. Further, the clinical course of dermatomyositis almost paralleled the level of serum tumor markers and the clinical course of her recurrent breast cancer. These markers were useful for detecting the recurrence, following the metastatic disease, and monitoring her response to therapy.
Journal Article↗
Dystrophin abnormalities in polymyositis and dermatomyositis.
The expression of dystrophin in muscle biopsies from nine cases of polymyositis, ten cases of juvenile dermatomyositis and three adults with dermatomyositis was studied by Western blot analysis and immunocytochemistry. Five antibodies corresponding to different N- and C-terminal regions of the dystrophin gene were used. Sixteen of the 22 cases (73%) showed an abnormality in the expression of dystrophin on Western blot analysis, either with a reduced molecular weight protein or a reduced amount. Immunostaining was abnormal in 11 out of 19 cases (58%) and showed varying degrees of discontinuity or loss of sarcolemmal staining. Immunolabelling of these areas with antibodies to beta-spectrin was normal implying that the changes were not caused by a loss of the sarcolemma. These results show that secondary changes in the expression of dystrophin can occur in the absence of an abnormality in the corresponding gene and that dystrophin cannot be used in isolation as a diagnostic marker for muscular dystrophy.
Adolescent↗
Transient elevation of the tumor markers CA 15-3 and CASA as markers of interstitial lung disease rather than underlying malignancy in dermatomyositis sine myositis.
A 37-year-old woman with severe interstitial lung disease associated with dermatomyositis sine myositis is reported. A thoracoscopic lung biopsy revealed organizing diffuse alveolar damage. Significantly elevated serum levels of the tumor markers CA 15-3 and CASA (cancer-associated serum antigen) were detected, but no evidence of an underlying malignancy (including breast and ovarian) was found on serial clinical and radiologic examinations. These levels gradually normalized as the interstitial lung disease responded to a combination of cyclophosphamide and corticosteroids. The use of the CA 15-3 and CASA assays to measure serum levels of the highly glycosylated, high-molecular-weight mucin MUC1 in interstitial lung disease has not been previously described. Clinicians should therefore be aware that elevation of these tumor markers may reflect the presence of interstitial lung disease rather than an underlying malignancy in patients with dermatomyositis, especially if the levels normalize after successful treatment of the lung disease.
Journal Article↗
Endothelial cell activation and neovascularization are prominent in dermatomyositis.
BACKGROUND: While vascular and immune abnormalities are common in juvenile and adult dermatomyositis (DM), the molecular changes that contribute to these abnormalities are not clear. Therefore, we investigated pathways that facilitate new blood vessel formation and dendritic cell migration in dermatomyositis. METHODS: Muscle biopsies from subjects with DM (9 children and 6 adults) and non-myositis controls (6 children and 7 adults) were investigated by immunohistochemistry using antibodies that recognize existing (anti-CD146) and newly formed blood vessels (anti-alphaVbeta3) and mature dendritic cells (anti-DC-LAMP). Blood vessel quantification was performed by digitalized image analysis. Additional muscle biopsies from subjects with adult DM and non-myositis controls were used for global gene expression profiling experiments. RESULTS: A significant increase in neovascularization was found in muscle biopsies of DM patients; neovascularization (alphaVbeta3 positive capillaries and vessels per muscle fiber) was much higher in juvenile than in adult DM patients (control vs juvenile DM: Mean +/- SE: 0.06 +/- 0.01 vs 0.6 +/- 0.05; p < 0.0001 and control vs adult DM: Mean +/- SE: 0.60 +/- 0.1 vs 0.75 +/- 0.1; p = 0.051). Gene expression analysis demonstrated that genes that participate not only in angiogenesis but also in leukocyte trafficking and the complement cascade were highly up regulated in DM muscle in comparison to age matched controls. DC-LAMP positive dendritic cells were highly enriched at perivascular inflammatory sites in juvenile and adult DM patients along with molecules that facilitate dendritic cell transmigration and reverse transmigration (CD142 and CD31). CONCLUSION: These results suggest active neovascularization and endothelial cell activation in both juvenile and adult DM. It is likely that close association of monocytes with endothelial cells initiate rapid dendritic cell maturation and an autoimmune response in DM.
Journal Article↗
An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM).
OBJECTIVE: To develop revised criteria for the diagnosis of juvenile dermatomyositis (JDM) using an international consensus process. METHODS: An initial survey was circulated to members of the Network for JDM and the Paediatric Rheumatology International Trials Organisation (PRINTO). Each individual was asked to identify those criteria that were felt to be most helpful in the diagnosis of classical JDM. A second survey was derived from these results and used to rank these proposed criteria in order of their importance and usefulness in clinical practice. RESULTS: The first survey had a response rate of 49.8% (118 individuals) from 92 centres in 32 countries. All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. Muscle biopsy, magnetic resonance imaging (MRI) and changes on the electromyogram (EMG) were deemed important diagnostic criteria. Other criteria, including myositis-specific or -related antibodies, nailfold capillaroscopy, factor VIII-related antigen, muscle ultrasound, calcinosis and neopterin, were used by 35.3% of respondents. Seventy-eight respondents to the first survey (66%) responded to the second survey. Typical MRI and muscle biopsy changes were rated by all to be the most useful clinically relevant diagnostic criteria after proximal muscle weakness, characteristic skin rash and elevated muscle enzymes. These were followed by myopathic changes on EMG, calcinosis, dysphonia and nailfold capillaroscopy, which were ranked equally. CONCLUSION: This process identified nine criteria that clinicians felt to be helpful or important in the diagnosis of JDM. A further process of refinement and validation is necessary to agree an internationally acceptable, clinically usable set of diagnostic criteria.
Biomarkers↗
Dermatomyositis without muscle weakness. Long-term follow-up of 12 patients without systemic corticosteroids.
BACKGROUND AND DESIGN: We retrospectively studied 12 patients with dermatomyositis (DM) with at least one laboratory criterion of muscle involvement but without muscle weakness. We reported their outcome without systemic corticosteroid treatment and searched for distinctive baseline data and associated neoplasia among a group of 29 patients with classic DM. RESULTS: After a mean follow-up of 5.3 years, complete or partial skin improvement occurred in nine patients with DM without muscle weakness, and muscle enzyme levels were normalized in all. When compared with the classic DM group, no statistical differences were found for associated neoplasia or for any baseline data studied except, as expected, for muscle parameters. CONCLUSIONS: The lack of distinctive baseline data between DM without muscle weakness and classic DM suggests that there is a continuum from amyopathic to classic DM. However, DM without muscle weakness is a clinical entity with respect to favorable long-term prognosis. Unless frank muscle weakness develops, treatment with systemic corticosteroids can be avoided.
Adolescent↗
Gingival telangiectases: an underappreciated physical sign of juvenile dermatomyositis.
BACKGROUND: MEDLINE searches (1966-June 1969) failed to identify references that give detailed descriptions of the oral manifestations of dermatomyositis (DM). However, several reports predating MEDLINE provided more complete descriptions of oral lesions associated with DM. OBSERVATIONS: We describe 5 cases of juvenile DM with oral manifestations, primarily in the form of gingival telangiectases. These findings are compared with those descriptions found in earlier reports. CONCLUSIONS: Oral lesions in juvenile DM have rarely been reported. Mucous membrane involvement associated with DM may include telangiectases, edema, erosions, ulcers, and leukoplakia-like areas. In cases of DM, gingival telangiectases likely represent an underappreciated diagnostic finding analogous to nail-fold telangiectases.
Child↗