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At least 19 recordsLinked to original sources
PubMed2003

Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis.

This study investigated interstitial pneumonia associated with amyopathic dermatomyositis, dermatomyositis and polymyositis, paying particular attention to muscular and/or cutaneous manifestations and their chronology relative to lung involvement. Patients included four males and 13 females, aged 51.7+/-10.8 yrs, who had surgical lung biopsy. Diagnoses included dermatomyositis (10 patients), polymyositis (four patients) and amyopathic dermatomyositis (three patients). Solitary respiratory manifestations preceded the onset of any skin or muscle disease in four cases (24%). Reticular and ground glass opacities were the most frequent computed tomography (CT) findings. Pathological review showed nonspecific interstitial pneumonia (eleven, 65%; cellular, two; cellular and fibrotic, five; fibrotic, four), usual interstitial pneumonia (two), organising pneumonia (two), lymphocytic interstitial pneumonia (one), and unclassifiable interstitial pneumonia (one). Nonspecific interstitial pneumonia was the most common histological pattern of interstitial pneumonia in patients with amyopathic dermatomyositis (three of three) and in patients with respiratory symptoms as the initial clinical manifestation of the connective tissue disease (three of four). Survival at 5 yrs was 50%. This study shows the clinician should remain alert to potential muscular or cutaneous manifestations whenever a pathological diagnosis of nonspecific interstitial pneumonia is made.

Adult↗
PubMed1986

Prospective study of familial canine dermatomyositis. Correlation of the severity of dermatomyositis and circulating immune complex levels.

Familial canine dermatomyositis in collie dogs is a newly recognized spontaneous disease that resembles dermatomyositis in children. A litter of 9 collie dogs was studied from birth to 7.5 months of age. The onset and severity of dermatitis and myositis correlated with elevated serum levels of circulating immune complexes (CICs) and IgG. The immunoglobulin component of the CICs consisted principally of IgG. All dogs developed elevated levels of CIC before or concurrent with the onset of dermatitis. Myositis developed later. The CIC tended to peak between 14-18 weeks of age in all dogs except the most severely affected dog, in which the CICs continued to increase to 238 micrograms/ml (controls 30 micrograms/ml) at 7 months of age. In the moderately affected dogs the CICs tended to stabilize at the levels reached at 14-18 weeks, and in the mildly affected dogs the CICs tended to decrease to normal levels after 14-18 weeks. Although the dogs had electromyographic and repetitive nerve stimulation abnormalities, the abnormalities did not correlate with severity of dermatomyositis or degree of elevation of CICs or IgG. Necropsy at 7.5 months of age revealed that all dogs had myositis and 8 of 9 had dermatitis. Except for 1 dog, the severity of dermatomyositis correlated positively with higher levels of CICs. A strong positive association between elevated levels of CICs and IgG, but not IgM or IgA, was generally present. Elevated levels of CICs appear to be involved in the mechanisms that control the development, severity, and progression of dermatomyositis in collie dogs.

Animals↗
PubMed1993

Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis?

BACKGROUND: Dermatomyositis sine myositis may have various connotations. Controversy exists as to nomenclature, degree of evaluation required, therapy, and course (e.g., does true dermatomyositis of the skin only exist?). OBJECTIVE: The purpose of this study was to assess prospectively patients with the clinicopathologic features of dermatomyositis and normal muscle enzyme serum levels to determine their course in terms of the onset of muscle disease. METHODS: Thirteen patients were studied by complete history and clinical examination, laboratory studies, electromyography, and skin and muscle biopsy. They were observed for 1 to 6 years. RESULTS: Patients were classifiable into three groups: (1) cutaneous changes only, (2) cutaneous changes only at baseline with subsequent development of myositis, and (3) cutaneous changes with normal muscle enzyme serum levels at baseline but with myositis demonstrated by electromyography and/or muscle biopsy specimens. CONCLUSION: Significantly different prognostic and therapeutic implications are present in patients with dermatomyositis with normal muscle enzyme serum levels depending on the results of electromyography, muscle biopsy, and clinical observation.

Adolescent↗
PubMed2002

Dermatomyositis in two siblings and a brief review of familial dermatomyositis.

Juvenile dermatomyositis is an uncommon autoimmune disease with classic heliotrope discoloration of the eyelids, erythematous skin rash of joints, and proximal muscle weakness. It is most frequently sporadic and only rarely familial. We present juvenile dermatomyositis in a 5-year-old brother and a 3 1/2-year-old sister, both are very responsive to corticosteroids. Familial dermatomyositis can occur in different family members, and even dermatomyositis and polymyositis can coexist in the same family.

Child, Preschool↗
PubMed1999

Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis.

This review will focus on the work that has been reported over the past year attempting to better characterize the overall clinical significance of the cutaneous manifestations of dermatomyositis (DM). It will be organized under two headings--amyopathic dermatomyositis (ADM) and classic dermatomyositis (CDM). The distinction between these two clinical phenotypes of DM is the absence of clinically-evident muscle disease in ADM; to date it has not been possible to distinguish the cutaneous manifestations of ADM and CDM. The term hypomyopathic DM is introduced to distinguish those patients with the hallmark skin changes of DM and no clinical evidence of muscle disease who are found to have subclinical evidence of myositis upon electrophysiologic, histopathologic, and/or radiologic evaluation. Recent issues that have been raised concerning the definition, classification, epidemiology, etiopathogenesis, clinical features, treatment, differential diagnosis, and prognosis of ADM and CDM are discussed. In view of the irrefutable evidence documenting the existence of ADM for 20 years or more, the term idiopathic inflammatory dermato-myopathies may be more appropriate for this group of clinical disorders than the currently used designation, idiopathic inflammatory myopathies.

Dermatomyositis↗
PubMed1991

Amyopathic dermatomyositis (dermatomyositis siné myositis). Presentation of six new cases and review of the literature.

We report six patients with the classic cutaneous findings of dermatomyositis who did not develop clinical or laboratory evidence of muscle disease for at least 2 years after onset of their skin manifestations. Such patients represent 11% of our total experience with dermatomyositis patients during a 15 year period. All six patients had Gottron's paules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. In addition, all complained of pruritus and photosensitivity. None of the patients had evidence of malignancy. Each of five patients treated with oral corticosteroids for their cutaneous disease had marked improvement and did not develop myositis. These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic of this disease and challenge the commonly held notion that muscle disease always develops within 2 years of onset of skin disease.

Dermatomyositis↗
PubMed1997

Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis.

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. These patients showed generalized follicular hyperkeratosis and diffuse thickening of the palms and soles. Histopathological findings consisted of keratotic plugging of the follicular infundibulum and features of erector pili myositis. We report on an 18-year-old woman with dermatomyositis. The diagnosis was established by characteristic enzymatic alterations, electromyographic pattern of myositis and the findings in a muscle biopsy, although the patient had no evidence of muscular weakness during a follow-up of 14 years. She developed an erythematosus and squamous eruption associated with diffuse palmoplantar keratoderma. Histopathological features consisted of a papillomatous epidermis with spicules of compact eosinophilic hyperkeratosis over the tips of papillae that were not related to hair follicles. Pityriasis rubra pilaris-like eruption seems to be a characteristic although uncommon cutaneous manifestation in dermatomyositis.

Adolescent↗
PubMed2002

Magnesium abnormalities of skeletal muscle in dermatomyositis and juvenile dermatomyositis.

OBJECTIVE: To characterize abnormalities in magnesium levels in the muscles of patients with dermatomyositis (DM) and juvenile dermatomyositis (JDM) and to evaluate the beneficial effects of prednisone and immunosuppressive therapy in elevating free magnesium (Mg(2+)) and ATP-bound magnesium (Mg-ATP). METHODS: The study groups consisted of 12 adult patients with DM and 10 juvenile patients with JDM. The 2 control groups were 11 normal adults and 6 healthy children. Levels of total ATP in the quadriceps muscles of the subjects were determined during rest, exercise, and recovery, using noninvasive P-31 magnetic resonance spectroscopy (MRS). Concentrations of the biologically active free Mg(2+) and the enzymatically active Mg-ATP complex were determined from the spectroscopy data by calculation of the chemical shifts of the beta-phosphate peak of ATP. RESULTS: Mg-ATP levels in DM and JDM myopathic muscles were at least 37% lower than those in normal muscles during rest, exercise, and recovery from exercise (P < 0.0005). Free Mg(2+) levels were normal in DM and JDM myopathic muscles at rest, but were significantly lower than control values during exercise and recovery (P < 0.029 and P < 0.005 for DM and JDM, respectively). Prednisone and immunosuppressive therapy partially reversed the magnesium abnormalities, as evidenced by elevation of the levels of Mg-ATP and free Mg(2+). CONCLUSION: Low levels of Mg-ATP and free Mg(2+) are concordant with weakness and fatigue observed in DM and JDM patients. Immunosuppressive therapy alleviates, in part, the magnesium deficits in the diseased muscles. Therefore, Mg-ATP and free Mg(2+) may play a significant role in the pathophysiology of these diseases.

Adenosine Triphosphate↗
PubMed1992

Dermatomyositis pemphigoides: a case with coexistent dermatomyositis and bullous pemphigoid.

We describe a patient with coexistent dermatomyositis and bullous pemphigoid; both appeared within a few weeks. Protein blotting showed binding of the patient's serum to the classic 220 kd bullous pemphigoid antigen. Because of the close temporal association of the two disorders, we believe that they are almost certainly etiologically linked. One possibility is that exposure of basement membrane antigens by dermatomyositis led to exposure of bullous pemphigoid antigen and subsequent antibody formation.

Aged↗
PubMed1999

A case of dermatomyositis that developed after delivery: the involvement of pregnancy in the induction of dermatomyositis.

A relationship between dermatomyositis (DM) and pregnancy has rarely been documented, and most cases have been reported from the viewpoint of the management of high-risk pregnancy. We report a patient with DM which developed after the delivery of a healthy infant. This case, with support from a literature review, suggests that pregnancy could be a trigger for the development of DM. Furthermore, it is suggested that there are at least two types of pregnancy related DM: in one type, the disease activity is provoked during pregnancy and tends to improve after delivery, while the other type (including the present case) has onset in the postpartum period.

Adult↗
PubMed1992

Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis.

We conducted a statistical review of 114 cases of dermatomyositis (DMS) treated primarily at the Department of Dermatology at Nagoya University Hospital over 27 years from 1965 to 1991 in order to determine the primary characteristics of juvenile DMS with the following results. 1) Juvenile DMS was found slightly more often in males than females; the male-to-female ratio was 1.4:1. Therefore, unlike adult DMS with its preponderance of females, there was no clear gender predominance. 2) Muscular manifestations tended to follow the appearance of cutaneous manifestations, but the frequency of minor muscular manifestations was high over the entire course of the disease. 3) Laboratory findings showed increases in serum aldolase and serum creatinine kinase with significant frequency when compared with adult patients (p < 0.01 and p < 0.05, respectively). Elevated serum aldolase most often occurred prior to or at the time of the appearance of muscular manifestations, suggesting its usefulness in early diagnosis. The positive rates for the antinuclear antibody on HEp-2 cells and anti-DNA antibody were significantly lower in children than in adults (p < 0.001 and p < 0.05, respectively). 4) There were no cases of juvenile DMS complicated by malignant tumors, interstitial pneumonia, or pulmonary fibrosis. There were also no deaths, and the rate of "remission or improvement" was significantly higher than in adult DMS cases (p < 0.05). Adult cases which remained the same or worsened usually presented with intractable muscular manifestations. In children, however, the cutaneous manifestations were more difficult to treat.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1976

[Dermatomyositis with spinulosis (Wong type dermatomyositis). Histologic study. Attempt at pathogenic interpretation].

The authors present a case of dermatomyositis with generalised spinulosis of pilose pityriasis rubra (lichen ruber acuminatus) type, giving a highly characteristic appearance of linear verruciform spinulosis of the back of the hands. They describe the histological lesions and feel that this spinulosis is probably due to marked degenerative smooth muscle myositis of the erector pili muscles. They recall the earlier cases of O'Leary and Christianson and above all those of Wong. The case reported is the first in a child.

Child↗
PubMed1991

[Juvenile dermatomyositis--statistical observation of 105 patients with dermatomyositis].

Statistical observation about the clinical items of 105 patients with dermatomyositis (DMS) attending Department of Dermatology, Nagoya University Hospital and some hospitals in Aichi Prefecture, during the year of 1965 to 1989, was carried out. Clinical features of the patients with juvenile DMS obtained from the observation were as follows. 1) The male:female ratio was 1.3:1 in juvenile DMS. The evident difference such as the predominance of females in adult DMS was not found. 2) The tendency that cutaneous manifestations usually preceded muscular manifestations was observed, and the muscular manifestations except severe symptoms were seen with a high incidence throughout the entire clinical course. 3) In the laboratory examinations, the incidence of elevation of serum aldolase concentrations in children was significantly higher than that in adults (p less than 0.05). Serum aldolase concentrations were usually elevated at onset or prior to the onset of muscular manifestations. Therefore the measurement of serum aldolase levels was considered to be useful for early diagnosis of juvenile DMS. The positive rate of antinuclear antibody in children was significantly lower than that in adults (p less than 0.001). 4) None of the children had any complications such as malignant tumors, interstitial pneumonia and pulmonary fibrosis, and none of them died. With regard to the outcome, the incidence of "remission or improvement" in children was significantly higher than that in adults (p less than 0.05). In the group of "same or worse", the children mainly had the cutaneous manifestations which were difficult to treat, compared with the adults. These results suggest that juvenile DMS may be a different disorder, probably a syndrome, from adult DMS.

Adolescent↗