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PubMed2006

Type I interferon-associated skin recruitment of CXCR3+ lymphocytes in dermatomyositis.

BACKGROUND: Dermatomyositis (DM) is an autoimmune disease of unknown origin affecting skin and muscles. Infiltrating autoreactive T lymphocytes are thought to play an important pathogenetic role, but it is unclear which mechanisms are involved in the recruitment of these cells. Recent studies provided evidence that a type I interferon (IFN)-driven immune response, including the recruitment of T cells via IP10/CXCR3 interactions, might be important for the generation of skin lesions of cutaneous lupus erythematosus (CLE), an autoimmune disease that shares some clinical and histopathological features with DM. We hypothesized that a similar mechanism might also be involved in the pathogenesis of DM skin lesions. METHODS: Skin biopsies of 23 donors (11 DM, 5 healthy controls, 7 CLE controls) were analysed by immunohistochemistry using monoclonal antibodies against CD3, CD4, CD8, CD20, CD68, CD123, the chemokine receptor CXCR3 and its ligand IP10/CXCL10, and the myxovirus-resistance protein A (MxA)-protein, which is a specific marker for type I IFNs. RESULTS: We detected strong expression of the MxA protein in all DM skin biopsies, indicating involvement of type I IFNs. Expression of MxA was closely associated with expression of the interferon-inducible protein IP10/CXCL10 and the recruitment of CXCR3+ lymphocytes. Plasmacytoid dendritic cells appear to be an important source of type I IFNs in DM. DISCUSSION: Our results support the hypothesis that lesional type I IFN signalling, induction of IP10 expression, and recruitment of potentially autoreactive T cells via IP10/CXCR3 interaction are involved in the pathogenesis of DM skin lesions.

Adult↗
PubMed1990

Relationship between capillary and muscle damage in dermatomyositis.

To assess the pathogenetic importance of capillary damage and its relationship with degenerating muscle fibers in dermatomyositis (DM), an electron microscope study of eight muscle biopsy specimens (adult and juvenile forms) and seven muscle specimens from patients with other neuromuscular diseases was conducted. There was a 49% reduction of capillaries in the muscle specimens of DM patients. Capillary damage also was more frequent in the DM group than in control group (p less than 0.001). We found a striking relation between capillary and muscle damage in the DM group (p less than 0.002) but not in the control group. The diagnostic value of undulating tubules within endothelial cells is also discussed.

Adolescent↗
PubMed1996

Juvenile dermatomyositis.

Juvenile dermatomyositis (JDMS) is a chronic inflammatory condition characterized by muscle weakness and a distinctive rash caused by underlying vasculopathy. Long-term complications include subcutaneous and muscular calcification, contractures and in some cases the gradual development of a second connective tissue disease. Early aggressive treatment with systemic immunosuppressants and other agents such as intravenous immunoglobulin (IVIG) reduces mortality and morbidity.

Calcinosis↗
PubMed1997

Characteristics of juvenile dermatomyositis in Japan.

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM, Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10,000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.

Child↗
PubMed1990

Management of fibrosing alveolitis with polymyositis dermatomyositis.

A 38-year-old man with polymyositis-dermatomyositis developed acute life-threatening fibrosing alveolitis. We report a two year follow-up of this patient, during which control of alveolitis was obtained using prednisolone, and was maintained with the addition of cyclophosphamide. Relapse of myositis responded to increased prednisolone. Serial measurements of vital capacity and transfer factor assisted management of alveolitis, but changes in vital capacity preceded changes in transfer factor, and were more convenient to perform during frequent outpatient review.

Adult↗
PubMed2004

Spontaneously remitting dermatomyositis.

Dermatomyositis (DM) is an inflammatory myopathy of skeletal muscle with characteristic cutaneous findings. It is a rare disorder with a bimodal age distribution that affects almost twice as many women as men. One category of DM, normal-enzyme DM, is characterized by cutaneous changes only at baseline, normal serum muscle enzyme levels and myositis demonstrated by electromyography (EMG) and/or muscle biopsy specimens. Typically, patients with normal-enzyme DM progress to severe muscle involvement and require systemic corticosteroid therapy. The patient we report has normal-enzyme DM confirmed by serial serum enzymes, EMG, and skin and muscle biopsies but is unique in that she never experienced progression of muscle weakness although muscle involvement was documented histologically and by EMG. Follow-up examination after 1 year revealed near-complete resolution of cutaneous involvement after topical therapy and no evidence of muscle weakness.

Aged↗
PubMed1982

Quantitative electromyography in polymyositis and dermatomyositis.

Twelve patients with polymyositis or dermatomyositis were examined repeatedly during the course of the illness by muscle strength tests, serum enzyme determinations, frequency analysis of the electromyographic signal, and estimations of the spontaneous activity. All methods can be used to follow the course of the disease. Increasing muscle strength was correlated to decreasing serum enzyme values, decreasing spontaneous activity, and decreasing proportion of high-frequency components in the EMG signal, except during the initial period of treatment, when a temporary increase in high-frequency components was recorded.

Adolescent↗
PubMed1979

Dermatomyositis with universal calcinosis. A histopathological and electron optic study.

Biopsies from a 5-year-old girl with dermatomyositis and universal calcinosis were studied histopathologically and by means of electron optical techniques. The dermis was infiltrated by fibroblasts, lymphocytes and plasma cells. In the lower part of the dermis, dense basophilic areas were present. In the vicinity of these areas irregular elastic fibers appeared. In the electron microscope these elastic fibers appeared mineralized to varying degrees. The mineral part was identified as calcium apatite (either calcium-hydroxy-apatite or calcium fluoroapatite) by X-ray microanalysis and selected area diffraction. No calcification was observed outside the elastic fibers.

Calcinosis↗
PubMed1985

Direct and indirect immunofluorescent findings in dermatomyositis.

Three skin biopsies (proximal nailfold, extensor forearm and buttock) and serum samples were studied by direct and indirect immunofluorescence, immunodiffusion and radioimmunoassay techniques in 6 patients with dermatomyositis. A variety of serologic and immunopathologic abnormalities was observed in the patients. Three of 6 patients had antinuclear antibodies (ANA) of a speckled pattern mixed with a peculiar dot pattern. Epidermal intercellular deposits of immunoglobulins were seen in the nailfold biopsies of 2 patients; one of them also had IgG deposits in the cytoplasm of epidermal cells. Forearm and buttock biopsies were negative.

Adolescent↗
PubMed1986

Ultrastructural study of calcinosis universalis with dermatomyositis.

Calcinosis universalis associated with dermatomyositis occurred in a 58-year-old woman. Tissues removed from the sublingual region in the patient were studied by ordinary microscopy, electron microscopy, and an electron-microanalytic method. The calcified materials were distributed on collagen fibers and seemed to have a relationship with foci of fibrinoid degeneration. Moreover, globular and/or membranous structures, considered to originate from the degenerate cells of the stroma, were observed in these calcified zones. Some of them contained electron-dense materials. Therefore, the globular and/or membranous structures were thought to be concerned with initial calcification in this case. Furthermore, irregular bone tissue was formed adjacent to the calcified masses. In addition, the calcified materials were identified by X-ray diffraction examination and electron microscopy as hydroxyapatite.

Calcinosis↗
PubMed1997

The immunofluorescent profile of dermatomyositis: a comparative study with lupus erythematosus.

We have demonstrated a role for microvascular injury mediated by the membrane attack complex of complement (C5b-9) in the genesis of cutaneous lesions of dermatomyositis (DM) (1). The purpose of this study is to revisit the immunofluorescent (IF) profile of DM, to further investigate the role of C5b-9 in the pathogenesis of cutaneous lesions, and to see if any features of the IF profile reliably distinguish DM from LE. Lesional skin biopsies from 24 patients with clinical findings characteristic of DM were received in formalin and in Michel's transport medium. Conventional light microscopy, and IF studies with antibodies monospecific for IgG, IgA, IgM, C3, fibrin and C5b-9 were performed. The control group comprised biopsies from 31 patients with well-documented LE. A positive lupus band test (LBT) correlated highly with a diagnosis of LE, with a sensitivity of 64.5% and a specificity of 95.6% (p=0.001). The LBT was most sensitive in the setting of DLE and SLE and was least sensitive in the setting of SCLE. The finding of vascular C5b-9 deposition correlated with a diagnosis of DM versus LE (p=0.001) although the false positive rate was 21.4%. The false negative rate was reduced when vascular C5b-9 was seen in the absence of antibodies to Ro, La, or RNP. While a negative LBT correlated with a diagnosis of DM (p=0.001), the specificity was only 64.5%. However, when it was seen in concert with C5b-9 along the DEJ, specificity was increased to 80.6% (p=0.001). The presence of C5b-9 in vessels and along the DEJ in concert with a negative LBT was predictive of DM (p=0.001) with a specificity of 93.5%, sensitivity of 78.3%, a false positive rate of 10% and a false negative rate of 14.7%. The combination of a negative LBT, vascular C5b-9 deposition and negative serology for Ro, La, and RNP was a predictor of DM versus LE with a sensitivity of 90.5%, a specificity of 96.8%, a false positive rate of 5% and a false negative rate of 6.2% (p=0.001). The IF profile of DM in lesional skin comprises a negative LBT, deposition of C5b-9 within vessels and along the DEJ, and variable keratinocyte decoration for IgG and C5b-9. The most statistically powerful predictor of DM is the combination of a negative LBT with vascular C5b-9 deposition and negative serology for antibodies to Ro, La, Sm, and RNP. Demonstration of a negative LBT in all but 1 case of DM suggests that the DEJ is not a primary site for antigen-antibody interaction. We postulate that the aforementioned IF findings reflect humorally mediated injury of endothelium and keratinocytes, effected by C5b-9.

Adult↗
PubMed2005

Evaluation of oral manifestations and masticatory force in patients with polymyositis and dermatomyositis.

BACKGROUND: The polymyositis (PM) and dermatomyositis (DM) complex encompasses a heterogeneous group of acquired autoimmune skeletal muscle diseases called idiopathic inflammatory myopathies (IIM). Despite their histological and immunopathological differences, the end result of the affected muscles in all of these entities is the triad of chronic inflammation, fibrosis and the loss of muscle fibres. The aim of this study was to perform a complete analysis of the orofacial abnormalities in 34 patients with PM and DM. METHODS: Evaluation of subjective oral symptoms, measurement of whole resting saliva flow rate (WS) with the 'spitting method', a visual investigation of the oral soft tissue alterations, light- and electron microscopic analysis of the symptoms of capillary abnormalities or signs of focal infiltration in labial biopsy specimens were carried out. The number of decayed, missing and filled teeth (DMF-T) according to the WHO recommendations (1997), the periodontal probing depth (PPD), the plaque index (PI; Silness-Löe) and the gingival index (GI; Löe-Silness) were determined. For comparison with healthy controls the masticatory force (MF) and the force of the upper extremities' flexors were measured with a specially developed device. The SPSS version 11.0 for Windows software program, two-tailed Student's t-test and Mann-Whitney test were used to statistically analyse all data. Values were considered to be significant if P level was < or = 0.05. RESULTS: Nine patients complained of subjective xerostomia, 11 showed the signs of salivary hypofunction (WS < or = 0.1 ml/min). The most prominent symptom of the oral mucosa and perioral tissues was the presence of telangiectasia, detected in seven cases. Fibrosis of the minor salivary glands was found in 12 patients, interstitial-perivascular infiltration was detected in eight cases, periductal infiltration in one case. The findings on dental and periodontal conditions indicate, that the patients with IIM diseases had significantly higher DMF-T scores (24.06 +/- 7.04 vs. 19.54 +/- 8.93, respectively; P = 0.002), they had less remaining teeth in average (15 vs. 20; P = 0.002), compared with the control group. Their oral hygiene was significantly worse (PI was 1.46 +/- 0.75 and 0.73 +/- 0.54, P = 0.001). GI was significantly higher in the patients (1.27 +/- 0.60 and 0.66 +/- 0.56, respectively, P = 0.0003), although we could not demonstrate any difference in the severity of the periodontal destruction between patients and controls. Masticatory force was significantly weaker in the first molar region on both sides in the patient group (309 +/- 213 N and 113 +/- 146 N, P = 0.0016 for the right side; 315 +/- 239 N and 123 +/- 76 N, P = 0.009 for the left side), but only the left hands showed to be weaker in the patient group (77 +/- 27 N and 59 +/- 20 N, P = 0.04). CONCLUSION: In conclusion, the present study showed that, patients with IIM diseases have hyposalivation and mucosal alterations, mainly in the form of telangiectasia. They have increased prevalence of dental caries, which may be secondary to the hyposalivation. The masticatory and the upper extremity flexor forces indicate that the weakness of the masticatory muscles can manifest earlier than that of the arms.

Adult↗
PubMed2004

Dermatomyositis: comparative studies of cutaneous photosensitivity in lupus erythematosus and normal subjects.

PURPOSE: Cutaneous features of dermatomyositis (DM) strongly suggest that ultraviolet (UV) radiation plays an important role in the pathogenesis of the disease. However, the incidence and the nature of photosensitivity in this disorder have not been established. The aim of this study was to investigate the UVB (290-320 nm) minimal erythema dose (MED) in DM patients in comparison with those in lupus erythematosus (LE) and healthy controls. METHODS: Non-irradiated back skin of 75 Caucasians with skin types II and III according to the Fitzpatrick classification were present in three different subject groups and tested for photomanifestation on non-irradiated suprascapular back skin with an ETG-1 Erythemtester. The first group included 19 DM patients, the second 30 patients with LE, and the third 26 healthy control volunteers. The MEDs were determined 24 h after irradiation adjusted according to skin type. RESULTS: Nine of the 19 DM patients (47.4%) demonstrated reduced MEDs to UVB radiation. Seven DM patients (36.8%) had a history of increased cutaneous photosensitivity and four of these (21.1%) reported diseased aggravation after sun exposure. Both the DM and LE patient groups showed reduced MED to UVB radiation (P<0.05) compared with the control group (19.2%). Increased erythemal sensitivity to UVB irradiation was found more frequently in patients with systemic LE and cutaneous discoid LE, than in those with subacute cutaneous LE. CONCLUSION: DM patients, similar to those with LE, showed a significantly reduced MED to UVB irradiation compared with healthy persons.

Adolescent↗
PubMed1990

Herpes zoster in patients with polymyositis and dermatomyositis.

Twenty-two patients with polymyositis and dermatomyositis (PM-DM) were retrospectively studied with regard to development of herpes zoster. Herpes zoster occurred with high frequency in patients with PM-DM. The clinical courses of zoster infections were uneventful; no severe complications nor deaths occurred, and only one patient had post-therapeutic neuralgia. No specific therapy for this infection was necessary. Zoster tended to occur in the inactive stage of PM-DM. PM-DM patients with herpes zoster had a significantly higher incidence of antinuclear antibody. There seemed to be no relationship between steroid therapy and herpes zoster infection.

Adult↗
PubMed1995

[A case of toxoplasmosis with dermatomyositis].

We report a case of dermatomyositis (DM) in a 15-year-old female with toxoplasmosis after ingestion of raw bovine liver. Facial erythema and cervical lymphadenopathy preceded myalgia and muscle weakness of the extremities. The diagnostic criteria of DM was fulfilled because of symmetrical and proximal dominant muscle weakness, elevation of myogenic enzyme (CPK, GOT, LDH, myoglobin, aldorase), myogenic pattern of electromyogram, skeletal muscle biopsy showing interstitial myositis with mild destruction of muscle fiber, and facial erythema. Immunological findings showed IgG anti-toxoplasma antibody to be 1340 IU/ml and IgM to be 7.0 (Cut off index 0.7), suggesting acute toxoplasmosis. Treatment with prednisolone for DM and acetylspiramycin for toxoplasmosis was successful. Toxoplasmosis should be considered as a possibility in patients with myositis.

Adolescent↗
PubMed1995

Flow cytometric analyses of the lymphocyte subsets in peripheral blood of children with untreated active juvenile dermatomyositis.

Juvenile dermatomyositis (JDMS) is a vasculopathy affecting primarily skin and muscle. Although the etiology is unknown, immunopathogenetic mechanisms appear to play a role in both the susceptibility to the disease and its progression. We measured the percentage and absolute numbers of B cells and T-cell subsets in the peripheral blood of untreated JDMS patients with active early disease and compared the results with those obtained from a study of peripheral blood obtained from a heatlhy age-related control group. The absolute number of total lymphocytes in the peripheral blood of the JDMS patients was significantly lower (P < 0.002) than that observed in the healthy control population, with an associated decrease in the absolute number of all T-cell subsets. No concomitant decrease in the absolute number of B lymphocytes was observed in the JDMS patients. In contrast, the percentage of B lymphocytes and the T-helper/T-suppressor cell ratio were significantly higher in the JDMS group than in the control group (P < 0.001 and P < 0.002, respectively). Retrospective analysis of JDMS patients' serum samples obtained within 1 month of the flow cytometric evaluation indicated that 79% of the sera contained an antinuclear antibody and 46% had immunoglobulin G values above age-adjusted reference ranges. The increased percentage of B cells, the increased T-helper/T-suppressor cell ratio, the positive antinuclear antibody results, and the increased concentration of serum immunoglobulin suggest that humoral immune dysregulation may contribute to the pathogenesis of JDMS.

Acute Disease↗
PubMed2000

Decreased levels of CD54 (ICAM-1)-positive lymphocytes in the peripheral blood in untreated patients with active juvenile dermatomyositis.

Significant abnormalities are observed in the peripheral blood of juvenile dermatomyositis (JDM) patients with active disease. In this study, we confirm that there is a significant increase in the relative percentage of B lymphocytes in the peripheral blood of a group of untreated children with newly diagnosed active JDM compared to healthy children (P < 0.0001). In order to investigate if properties intrinsic to B cells contributed to their relative increase in JDM, the percentage of B cells expressing activation markers (CD23, CD25, CD54, and CD69) was measured and compared to pediatric controls. Compared to healthy children less than 10 years of age (not significantly different from the JDM group), the JDM patients had an increase in the proportion of lymphocytes expressing CD19 (B cells; P = 0.0017) and decreases in the percentage of lymphocytes that were CD3(-) CD16(+) and/or CD56(+) (NK cells; P = 0. 01) and CD3(+) CD8(+) (T suppressor/cytotoxic cells; P = 0.02). There were no significant differences in any of the B-cell activation markers assessed. Of note, the percentage of CD54(+) non-B lymphocytes (i.e., T cells and NK cells expressing CD54) was significantly lower in the JDM patients (25% +/- 5%) than in the "age-related" healthy control group (43% +/- 4%; P = 0.013). These results suggest the following for untreated children with active JDM: (i) the increase in the percentage of peripheral blood B cells is not due to intrinsic B-cell activation, and (ii) CD54/ICAM-1(+) non-B cells, CD8(+) T cells, and NK cells are being removed from circulation and may be participating in the pathophysiology of the disease.

Dermatomyositis↗
PubMed1976

Treatment of dermatomyositis in childhood.

Analysis of the response to corticosteroid therapy in a personal series of 8 consecutive cases of dermatomyositis in childhood shows that there are advantages in a moderate dosage, short-term treatment schedule, with gradual tapering of the dosage as soon as there is clinical improvement without waiting for full remission, and in trying to stop steroid therapy within six months rather than following the more prolonged regimen currently still in vogue. Clinical response is a more reliable guide to progress than serum enzyme levels. Review of published reports suggests that overtreatment with corticosteroids may be a factor in chronicity of the disease and failure of adequate long-term response.

Azathioprine↗