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PubMed1981

Muscular strength as an index of response to therapy in childhood dermatomyositis.

Dermatomyositis, an inflammatory disease of unknown etiology, causes diffuse symmetrical weakness and atrophy, muscular pain and tenderness, induration of muscles, and the tendency to develop contractures. The disease may follow a prolonged course which can best be managed with steroids and regulation of physical activity if there is an objective criterion for determining the extent of clinical involvement. In 6 children with dermatomyositis, quantitative muscular strength was compared with clinical evaluation of the state of the disease, serum enzyme levels, and other laboratory measures of systemic inflammation. Quantitative evaluation of ankle plantar flexor strength by the method of Beasley or handgrip force by the method of Mundale indicated that muscular strength provided a better criterion for the clinical status of the patient than any of the other laboratory tests studied. In dermatomyositis, the inflammation is equally great in distal and proximal muscles when tested quantitatively. These tests, when used together with enzyme levels and clinical evaluation, permit more effective management of dermatomyositis in children.

Adolescent↗
PubMedDate not supplied

[Familial neoplasms and childhood dermatomyositis].

Familial frequency of malignant neoplasms from 20 children with dermatomyositis was investigated and compared with 225 controls. Eight patients with dermatomyositis (40 per cent) had nine family members with a malignant tumour; this frequency is significantly higher than those found in the controls with juvenile rheumatoid arthritis (P less than 0.01), neoplasms (P less than 0.01), and a variety of diseases (P less than 0.05). This finding and the known association of neoplasm-dermatomyositis might suggest an hereditary predisposing factor, possibly a subtle inmune deficiency, common for tumours and dermatomyositis.

Child↗
PubMed1994

[Dermatomyositis and eclampsia. Analysis of a case and review of the literature].

Dermatomyositis is an inflammatory disease of unknown etiology whose association to pregnancy is uncommon. The impact of dermatomyositis with uncomplicated pregnancy while active is very high for the fetus without major maternal consequences. The monitoring of cases with pregnancy associated hypertension and dermatomyositis, must be performed independently for the mother and the fetus, since fetal prognosis is usually bad and does not modify with treatment. The case presented herein is to our knowledge the first with eclampsia and dermatomyositis which ended in a normal product delivered and the mother decreased.

Adult↗
PubMed1993

Differential diagnosis between fibrodysplasia ossificans progressiva and childhood dermatomyositis with calcinosis.

Both fibrodysplasia ossificans progressiva (FOP) and childhood dermatomyositis with calcinosis are rare diseases, and present with ossifying or calcifying processes. Six cases of FOP and one case of childhood dermatomyositis with calcinosis are studied. All six FOP patients had the typical digital anomalies and the characteristic ectopic bone formation starting from the trunk. Calcification in the case of childhood dermatomyositis occurred in the limbs. A carefully differentiated diagnosis between these two diseases is needed, because they share common clinical and radiologic features, but require different management. Delay in the diagnosis of FOP is common, although early recognition of FOP prevents a child from accidental or iatrogenic injury, which can precipitate ectopic ossification. Surgical removal of the ectopic bone or release of the contracture in three FOP patients was followed by rapid recurrence. Entrapment neuropathy and a mild myopathic pattern in two FOP patients, who underwent nerve conduction and electromyographic studies, were secondary to ectopic bone formation. One FOP patient received a computed tomography examination which showed basal ganglia calcification. No coexistence of FOP and childhood dermatomyositis with calcinosis was found.

Adolescent↗
PubMed1993

Xe-133 muscle blood flow in polymyositis and dermatomyositis.

The histologic picture of polymyositis or dermatomyositis is characterized by muscle fiber degeneration, which is assumed to be caused by a circulatory abnormality. To verify this assumption, we measured the muscle blood flow in patients with polymyositis (or dermatomyositis) and in normal subjects using the inert gas Xe-133. Sixteen patients and 30 age-matched normal volunteers were studied. Muscle blood flow was measured with Xe-133 dissolved in isotonic saline as described by Lassen et al. (1964) and Pozderac et al. (1975). Data were acquired in the frame mode after intramuscular injection (anterior tibial muscle) of 0.3 mCi of Xe-133 in 0.2ml of saline for 20 min. A computer routine was used to calculate the clearance half time (T 1/2) in min and flow rate (Q) in ml/100 gm tissue/min. The student's t test was used to test statistical significance. Our preliminary results suggest: 1. The muscle blood flow in patients with polymyositis and dermatomyositis was significantly lower than the corresponding normal control levels. 2. This findings may be due to decreased in muscle capillary 3. Further studies are warranted to obtain a conclusive understanding of the pathogenesis of polymyositis and dermatomyositis.

Adult↗
PubMed1993

[Intravenously-administered immunoglobulins as first-choice agent in juvenile dermatomyositis].

Dermatomyositis is an acquired disease characterised by symmetric predominantly proximal muscle weakness of the arms and legs, and misery. It may be associated with myalgia and there is often a characteristic rash. The mainstay of therapy is corticosteroids. Recently efficacy of intravenous immunoglobulin (IVIg) in chronic refractory dermatomyositis was reported. Because corticosteroids can cause serious side effects, we treated a seven-year-old girl suffering from dermatomyositis with IVIg as initial therapy. After two courses of IVIg infusions at a dose of 0.4 g/kg/day for five consecutive days, the patient made a rapid and complete recovery. This case shows that IVIg may be effective as initial therapy in patients with dermatomyositis. Whether IVIg is really a better treatment than corticosteroids should be investigated in a randomised study.

Biopsy↗
PubMedDate not supplied

Dermatomyositis and malignancy: case report and review of the Japanese literature.

The first reported association of dermatomyositis with malignancy was by Stertz in 1916, who described a patient with proximal muscle weakness, eyelid changes, and evidence of myositis on muscle biopsy as well as a coexisting gastric carcinoma. In the same year, Kankeleit described a patient with dermatomyositis and breast cancer--the seeds of a controversy were thus sewn. We report a female patient with multiple cancer who developed dermatomyositis and review the relevant Japanese literature. Our patient suffered from metachronous bilateral breast cancer and thyroid cancer. She underwent curative resection of all 3 tumors. Our experience suggests that clinicians should perform extensive screening of dermatomyositis patients to salvage those with occult cancer, although the issue of cost effectiveness also has to be considered.

Adolescent↗
PubMed1996

Membranocystic changes in the panniculitis of dermatomyositis.

Clinically apparent panniculitis is rare in dermatomyositis. The common histopathological findings are infiltration of lymphocytes, epithelioid cells and plasma cells in the fat lobules, along with varying degrees of fat degeneration and fibrosis. We report a 65-year-old woman with dermatomyositis who developed panniculitis with a characteristic histological change known as a membranocystic lesion. Although this change has been observed in various diseases affecting the subcutaneous fat tissue, it has rarely been reported in dermatomyositis. Dermatomyositis should be included in the diseases showing a membranocystic lesion.

Aged↗
PubMed1997

[A case of dermatomyositis with severe retinopathy in a patient who died of acute interstitial pneumonia].

Sight threatening ocular complications are rare in adult patients with dermatomyositis. We encountered a 52-year-old female with dermatomyositis who had severe visual disturbance and rapidly progressive intersitial pneumonia. She was admitted to our hospital because of skin erythema, general fatigue, mild fever, and severe bilateral visual disturbance. Rentinal hemorrhages, cotton wool spots, and macular edema were observed in her fundus at the first ophthalmic examination. A diagnosis of dermatomyositis was made because of the myogenic pattern of her electromyogram, elevation of serum creatine kinase, and skin lesions. Oral prednisolone treatment was started and the retinopathy was improved, but was complicated by acute interstitial pneumonia. The interstitial pneumonia was not respond to steroid pulse therapy with methylprednisolone, and the patient died of respiratory failure on the 47th day after the onset of visual symptoms. In adult dermatomyositis patients, the complication of severe retinopathy should be considered as a risk factor for rapid progress of interstitial pneumonia.

Acute Disease↗
PubMed2002

Drug-induced amyopathic dermatomyositis.

Amyopathic dermatomyositis is a rare condition. We describe the clinical and histopathologic findings of a 53-year-old woman who developed cutaneous lesions similar to those described in dermatomyositis after fibrate therapy for hypertriglyceridemia. Our patient fits the proposed criteria for amyopathic dermatomyositis, and after 8.5 years, muscular involvement has not been detected, although the dermatologic lesions are still present despite different treatments reported to treat this condition (steroids, antimalarials, methotrexate, dapsone). However, an initial good response has been observed with thalidomide, a drug used increasingly in the dermatologic field. As far as we know, this case is the first of drug-induced amyopathic dermatomyositis.

Journal Article↗
PubMed2006

The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr.

OBJECTIVES: To identify epidemiological, clinical and laboratory characteristics of juvenile dermatomyositis (JDM) in a national multi-centre cohort of patients, and to review recent changes in the understanding of management and prognosis in the light of these data. METHODS: All children with idiopathic inflammatory myositis recruited to the Juvenile Dermatomyositis National Registry and Repository (UK and Ireland) were included. Features at presentation, and later in disease, were assessed and evaluated. A total of 63 out of 175 children with a new diagnosis of myositis were recruited at the time of diagnosis and followed prospectively. Out of the 175 children, 122 diagnosed prior to 2000 were recruited retrospectively, with subsequent data collected prospectively. RESULTS: One patient died (0.7%), which is equivalent to one death per 465 patient years. Data were available at the time of analysis on 151 registered patients. The most common presenting features were characteristic rash, weakness, tiredness, Gottron's patches and myalgia. Muscle biopsy, magnetic resonance imaging and muscle enzymes were frequently, but not always, abnormal. Muscle enzymes and erythrocyte sedimentation rate were not useful markers of disease activity. CONCLUSIONS: The JDM National Registry and Repository captures data on a significant cohort of children with inflammatory myositis. The current study reports the largest European cohort of children with dermatomyositis to date. This powerful resource will help improve our understanding of this rare disease. Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy, and variable outcome of childhood myositis.

Adolescent↗
PubMed1979

Toxoplasmosis appearing to be dermatomyositis.

A case of toxoplasmosis occurred simultaneously with dermatomyositis in a 12-year-old boy. The patient was treated with sulfadiazine and pyrimethamine; within two weeks after initiating therapy, dramatic clinical improvement was noted. Several previous cases of toxoplasmosis occurring in association with polymyositis have been described in the literature. A serologic investigation for Toxoplasma infection might prove to be of value in establishing the cause of dermatomyositis and polymyositis. Moreover, in selected cases of dermatomyositis or polymyositis, treatment with sulfadiazine, pyrimethamine, and folinic acid may be a valuable alternative to the use of steroids and immunosuppressive agents.

Child↗
PubMed1979

Childhood dermatomyositis and polymyositis. Treatment with methotrexate and prednisone.

The conditions of three children with dermatomyositis and one child with polymyositis were treated for nine to 31 months with combined prednisone and intravenous methotrexate (1 mg/kg/wk) when prednisone alone was ineffective in controlling the disease or when there were substantial steroid-related toxic effects. All children showed a major clinical improvement within three months despite concomitant reduction of the prednisone dose. Three children completely recovered; one patient relapsed and died. The toxic effects of methotrexate included elevated liver transaminases (3/4), nausea (2/4), abdominal pain (2/4), bone pain (2/4), mild neutropenia (1/4), and mild pruritus (1/4). Intravenous methotrexate is an effective adjunct to steroid therapy in the treatment of steroid-resistant or life-threatening dermatomyositis-polyositis or dermatomyositis-polymyositis complicated by severe steroid-related effects.

Child↗
PubMed1998

Spontaneous abdominal hematoma in dermatomyositis.

Dermatomyositis is associated with a number of systemic manifestations and diseases. We present 2 patients with dermatomyositis, aged 11 and 50 years, who developed acute abdominal pain, both a result of spontaneous hemorrhage. Hemorrhage was detectable by physical examination in one and on computed tomography scan of the abdomen in the other. Both patients made a full recovery with supportive treatment. While the cause of the hemorrhage was uncertain, in 1 patient massive calcinosis of the abdominal wall was present, and trauma may have been the precipitant. Spontaneous abdominal hematoma is a cause of acute abdominal pain in patients with dermatomyositis, and surgery may be avoided if the diagnosis is recognized.

Abdominal Muscles↗
PubMed1981

Osteosarcoma arising in heterotopic ossification of dermatomyositis: case report and review of the literature.

A patient with dermatomyositis developed malignant transformation of the benign interfascial heterotopic bone. This patient had classic childhood dermatomyositis at the age of 3 years, and the disease was arrested after a one-year course of corticosteroid therapy. Extensive subcutaneous calcinosis cutis and deep interfascial calcinosis were the residua of the disease. Twenty-eight years later, the patient developed a high-grade osteosarcoma within the benign intermuscular heterotopic calcification and ossification that had been previously documented at the age of 8 years by a roentgenogram and at the age of 16 years by biopsy. This case represents the first report of the association of osteosarcoma and dermatomyositis, and possibly the first well-documented case of malignant transformation of benign heterotopic bone.

Adult↗
PubMed1982

Nailfold capillary abnormalities and clinical outcome in childhood dermatomyositis.

The nailfold capillary pattern was observed in a population of patients with childhood dermatomyositis. Distinctive nailfold capillary loop abnormalities were found in 11 of 19 childhood dermatomyositis patients and in none of 2 control populations (P less than 0.001). By a retrospective analysis of the childhood dermatomyositis patients, we found that the presence of nailfold capillary abnormalities correlates with more severe forms of the disease (ulcerative and chronic types), as opposed to limited type of disease. These changes occurred independently of disease activity or of cutaneous abnormalities.

Adolescent↗
PubMed1995

Lyme disease in a 74-year-old forest owner with symptoms of dermatomyositis.

We describe a 73-year-old forest owner with widespread erythema, myalgia, and proximal muscle weakness. The clinical signs and the results of electromyography, magnetic resonance imaging, and a muscle biopsy were consistent with dermatomyositis. However, serology was positive for Borrelia burgdorferi. More importantly, B burgdorferi DNA was detected in skin by polymerase chain reaction techniques, and spirochete-like organisms were detected in the muscle by silver staining. Lyme disease with muscle involvement can mimic or trigger dermatomyositis and should be considered in the differential diagnosis of dermatomyositis.

Aged↗
PubMed2003

Myokymia, neuromyotonia, dermatomyositis, and voltage-gated K+ channel antibodies.

A young woman presented with facial myokymia in association with dermatomyositis. There was no evidence of peripheral neuropathy. Needle electromyography showed prominent myokymic discharges and brief neuromyotonic discharges in addition to many small-amplitude, short-duration motor unit potentials. Myokymia and dermatomyositis both responded to immunosuppressive treatment. The presence of antibodies to voltage-gated potassium channels and the association with dermatomyositis indicated an autoimmune cause for myokymia, which may have been due to reversible peripheral nerve hyperexcitability.

Action Potentials↗