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[Spontaneous pneumomediastinum in dermatomyositis: a pathophysiological approach using imaging].

INTRODUCTION: Spontaneous pneumomediastinum is a rare complication of dermatomyositis. CASE REPORT: We report a case of pneumomediastinum with massive subcutaneous emphysema occurring in a female patient with dermatomyositis treated with cortico-steroids. CONCLUSIONS: Our case illustrates perfectly the mechanism of spread of air along the broncho-vascular structures and also explains the presence of pneumomediastinum in the absence of pneumothorax.

Dermatomyositis↗

[A case of dermatomyositis associated with prostatic carcinoma: a case report].

We report a case of dermatomyositis associated with prostatic carcinoma. A 69-year-old male was admitted to the Department of Internal Medicine with the chief complaint of general fatigue, appetite loss and facial anthema. Abdominal ultrasound demonstrated swollen periaortic lymph nodes and the margin of prostate was unclear. Prostatic carcinoma was suspected based on digital rectal examination, so he was admitted to our department. Serum prostate specific antigen level was 190 ng/ml. He was examined by a dermatologist because of deterioration of anthema. Dermatomyocitis was demonstrated by dermatoses (edema erythema at face, neck and limbs, nail fold thrombosis and poikiloderma), high serum level of creatine phosphokinase and a decrease in muscular strength (especially at the proximal musculus). There was no interstitial pneumonitis or malignancy of the digestive system. On needle biopsy of the prostate and quadriceps femoris muscle, prostatic carcinoma (poorly differentiated adenocarcinoma, Gleason score 5 + 5) and myositis were suspected. The stage of prostatic carcinoma was T4N1M1. The patient was treated by administration of diethylstilbestrol phosphate and prednisolone for prostatic carcinoma and dermatomyositis, respectively, but he died of multiple metastasis of the tumor 1 year and 5 months later. Dermatomyocitis is associated with malignancy more frequently than any other collagen disease. In Japan, it is frequently complicated by gastric, lung and mammory cancers, but rarely by prostatic carcinoma. To our knowledge, this is the fourth case of prostatic carcinoma associated with dermatomyocitis in Japan.

Adenocarcinoma↗

Therapeutic response to pentoxifylline and its active metabolites in dogs with familial canine dermatomyositis.

Ten dogs diagnosed with familial canine dermatomyositis based on clinical findings and skin biopsy results were treated PO with pentoxifylline (25 mg/kg twice daily) for 3 months. Blood and urine samples were collected biweekly for complete blood counts, serum chemistries, and urinalysis. Additionally, serum was analyzed for pentoxifylline and its two primary metabolites (metabolites 1 [M1] and 5 [M5]); physical examinations were performed; and skin lesions were photographed and subjectively scored biweekly. The median time to response (regrowth of hair) was 6 weeks (range = 4 to 10 weeks). Clinical chemistries, hematology, and urinalysis variables did not differ between the first sampling time and the final evaluation (Week 12). In general, pentoxifylline and its two metabolites were detectable only at the peak (3-hour) sampling time. M5 concentrations were the highest of the three compounds at each sampling time and were characterized by the least variability. Statistical differences in concentrations between Week 2 and Week 12 did not exist. Four of the 10 dogs were characterized as having a complete clinical response to treatment, and six dogs had a partial response. Based on these responses, 25 mg/kg every 12 hours appeared to be an effective beginning dosage of pentoxifylline for dogs with familial canine dermatomyositis. Because of the variability in disposition, including metabolite formation, monitoring of pentoxifylline concentrations may not offer a therapeutic advantage.

Administration, Oral↗

Dermatomyositis with tuberculous fasciitis.

A case of dermatomyositis with tuberculous fasciitis is described. A 69-year-old Japanese man treated with prednisolone for dermatomyositis developed erythema and bilateral swelling of arms and forearms. A diagnosis of tuberculous fasciitis was confirmed by the presence of acid-fast bacilli in a biopsy specimen and the growth of Mycobacterium tuberculosis from tissue cultures. The unusual presenting clinical features resulted in delayed diagnosis. We emphasize that tuberculous fasciitis should be considered in an immunocompromised patient who is treated with steroids, especially if the results of initial treatment are not satisfactory.

Aged↗

The expression of cyclooxygenase-1, cyclooxygenase-2 and 5-lipoxygenase in inflammatory muscle tissue of patients with polymyositis and dermatomyositis.

OBJECTIVE: To describe cyclooxygenase-1 (COX-1), cyclooxygenase-2 (COX-2) and 5-lipoxygenase (5-LOX) expression in muscle tissue in patients with idiopathic inflammatory myopathies (IIM) - dermatomyositis (DM) and polymyositis (PM) and to find out if any differences between affected and non-affected muscles detected by MRI exist. METHODS: Samples of muscle tissue from 7 patients with dermatomyositis (DM) and from 4 with polymyositis (PM) were obtained by needle biopsy from affected and non-affected sites distinguished by magnetic resonance imaging. In situ hybridization with antisense mRNA probes was employed to detect COX-1, COX-2 and 5-LOX mRNA. RESULTS: Expression of COX-1, COX-2, and 5-LOX mRNA was found in all samples - in the muscle cells, inflammatory cells and in vessels. COX-1 mRNA expression predominated in the inflammatory cells and vessels and was higher in affected than in non-affected sites detected by MRI (mean intensity 3.22+/-0.67 vs. 2.0+/-0.87; p = 0.0006). The expression of COX-2 mRNA was high mainly in inflammatory cells and/or vessels and was increased in MRI-detected affected tissues (3.5+/-0.88; 1.9+/-1.1; p = 0.003), as was the expression of COX-2 mRNA in muscle cells (2.1+/-1.0 vs. 1.3+/-1.0; p = 0.021). 5-LOX mRNA was largely expressed in muscle cells from MRI-detected affected sites and the signal intensity was higher in comparison with samples taken from non-affected tissues detected by MRI (3.22+/-0.7 vs. 1.67+/-0.7; p = 0.0007). CONCLUSION: Expression of COX-1, COX-2 and 5-LOX mRNA was observed for the first time in muscle tissues from IIM patients. This expression was increased in affected tissues detected by MRI, which may suggest a role of COX-1, COX-2, and 5-LOX in the pathogenesis of IIM.

Adult↗

Clinical case of the month. 3-year-old boy presenting with a hand rash. Juvenile dermatomyositis.

Of the diseases within the spectrum of the juvenile idiopathic inflammatory myopathies, juvenile dermatomyositis (JDM) is the most common. As the name implies, JDM affects the muscles and skin most commonly, but can involve other organ systems as well. Dermatologic manifestations often precede other signs and symptoms by months or even years and frequently are the primary reason the patient seeks medical attention. In the case presented here, a 3-year-old boy initially developed a hand rash that brought him to his primary care physician. By the time muscle weakness had developed, the patient had already been evaluated for dermatomyositis and therapy had been initiated. An understanding of these early clinical findings will enable physicians to make a timely diagnosis and commence therapy promptly in order to prevent life-threatening sequelae of the disease.

Child, Preschool↗

[Spontaneous remission of dermatomyositis which developed one month after normal delivery].

A case of dermatomyositis which developed one month after normal delivery and subsided spontaneously was reported. A 29-year-old woman gave birth to a healthy child. One month later, she noticed muscular pain and weakness of the upper extremities. On admission, there were diffuse edema of upper eyelids with heliotrope rash. The reddish skin rashes were observed on the extensor surfaces of the PIP and MP joints of fingers. Erythrocyte sedimentation rate was 29 mm/hr. The lactic dehydrogenase (LDH), SGOT, CK levels were 470 (normal 150 to 320 IU/l), 43 (normal 6 to 25 IU/l) and 317 (normal 21 to 110 IU/l) respectively. Autoantibodies to nuclear and cytoplasmic antigens were negative. Rheumatoid factor and anti-DNA antibody were negative. Thyroid function was normal. An electromyogram (EMG) demonstrated small amplitude short-duration polyphasic motor unit potentials. The muscle biopsy specimen from left upper arm showed degenerating muscle fibers and infiltration of inflammatory cells surrounding blood vessels. The skin biopsy revealed the presence of edema and perivascular infiltration of lymphocytes. Based on these clinical features and results of various diagnostic tests, a diagnosis of dermatomyositis was established. After the admission, muscle strength has improved dramatically and the CK returned to normal level without specific drug therapy. She has since been seen as an out patient, and complete remission lasted for two years up to date. Review of the literature disclosed that 13 cases of PM/DM which developed during pregnancy or postpartum have been reported including the present case. Detailed analysis showed that these patients were characterized by mild muscular diseases, rare occurrence of internal organ involvements and good response to steroid therapy. As our case, a spontaneous remission was also observed. Although the mechanism involved in occurrence of inflammatory myositis associated with pregnancy or delivery are not clarified, these patient indicated a presence of subset of PM/DM which do not require intensive drug therapy.

Adult↗

Combination of massive mucinosis, dermatomyositis, pyoderma gangrenosum-like ulcer, bullae and fatal intestinal vasculopathy in a young female.

Cutaneous mucinosis secondary to autoimmune collagen vascular disease is well recognized, but manifestation as cellulitis-like massive cutaneous mucinosis preceding dermatomyositis is unusual. Here we report a 21-year-old Taiwanese woman with a large, rapid onset, painful erythematous, edematous plaque, which histopathologically revealed septal panniculitis with fat necrosis and massive mucin deposition. Incapacitated muscle weakness of proximal extremities, generalized edema, heliotrope erythema, and Gottron's papules developed in a short period of time with high titers of serum muscle enzyme. Serological titers of ANA, anti-dsDNA, anti-ENA panels, and erythrocyte sedimentation rate, however, all showed unremarkable results. Diagnosis of dermatomyositis was confirmed by electromyographic findings of myopathy. As the disease progressed, large, deep cutaneous ulceration and vesiculobullous lesions also developed. In spite of aggressive treatment, the patient died 9 months after the disease onset, probably due to the complication of gastrointestinal ischemia and perforation.

Adult↗

Dermatomyositis and pregnancy. Case report and review of the literature.

Dermatomyositis is a rare disease complicating pregnancy. We treated a patient in whom two pregnancies were complicated by dermatomyositis. In the first of the two gestations, a mild form of the disease arose in the last trimester. In the second gestation, the disease was inactive following treatment with 0.3 mg/kg/day prednisolone. Both the mother and two children have since been doing well.

Adult↗

Hypercalcemia during the resolution of calcinosis universalis in juvenile dermatomyositis.

Dystrophic calcification is seen in more than 50% of children with juvenile dermatomyositis and tends to resolve spontaneously in some patients. Calcinosis universalis is the least common type of calcification seen and rarely regresses. We describe a boy with juvenile dermatomyositis and calcinosis universalis who developed hypercalcemia during spontaneous regression of dystrophic calcification. The treatment and possible mechanisms of this complication are discussed.

Calcinosis↗

[Chronic hemorrhagic cystitis induced by cyclophosphamide in dermatomyositis refractory to corticosteroid treatment].

Dermatomyositis was diagnosed at age 7 years to a fourteen year old female. The disease was unresponsive to high doses of prednisone, so she was treated with oral cyclophosphamide 0.8 mg.kg.day (25 mg.m2) but treatment was withheld after sixteen weeks because of hemorrhagic cystitis and changed to azathioprine for two years, which resulted in complete remission of dermatomyositis. Nevertheless silent microscopic hematuria persisted and chronic cystitis was demonstrated by cystoscopy and vesical biopsy at age 13 years. In this case chronic cystitis developed earlier and at lower doses of cyclophosphamide than in other previously reported cases.

Adolescent↗

[Scintigraphic findings of liver and spleen in dermatomyositis].

In 3 patients with dermatomyositis scintigraphic examination of liver and spleen revealed in every case diseases of the liver such as hepatoma, hepatomegaly and damage of the liver parenchyma. Therefore scintigraphic examination of liver and spleen in patients with dermatomyositis is suggested to exclude primary or secondary affections of these organs.

Aged↗

Dermatomyositis associated with the recurrence of transitional cell carcinomas and Kaposi's sarcoma.

A 76-year-old male was admitted to our hospital because of dermatomyositis (DM). Fourteen years earlier a transitional cell carcinoma of the bladder was diagnosed. Following repeated resections of the neoplasm, he was free of the disease for the last two years. Six weeks after starting treatment with steroids and azathioprine for DM, he has developed Kaposi's sarcoma of the skin which subsided upon discontinuation of the immunosuppressive drugs. Four months after the diagnosis of DM, a local recurrence of the bladder carcinoma was found. This patient illustrates the importance of a thorough search for neoplasms or recurrence in elderly patients with dermatomyositis. The case also emphasizes the need for caution in immunosuppressive treatment of autoimmune disorders in such patients. Still, discontinuation of these medications resulted in a significant improvement of the skin neoplasm.

Aged↗

[Staircase phenomenon in children with progressive muscular dystrophy and dermatomyositis].

The electrical and mechanical activity of the isometric twitch of flexor carpi ulnaris muscle during two per second indirect supramaximal stimulation for 90 sec was examined in 14 children with muscular distrophy and 8 children with dermatomyositis. The muscle electrical responses show no significant changes in the amplitude of its first negative phase. The first derivative of the dinamogram shows some of the following abnormalities in 8 of the examined children with muscular distrophy and in 2 of these with dermatomyositis: 1. Prolonged and increased negative staircase; 2. Insufficient or absent positive staircase potentiation. These abnormalities of the staircase phenomenon disclose disorders of the contractile function of the examined muscle.

Adolescent↗

[Two cases of amyopathic dermatomyositis with fatal rapidly progressive interstitial pneumonitis].

Two middle-aged women showed typical erythematous heliotrope eruption and Gottron's sign without any symptom of myositis. The patients were diagnosed as 'amyopathic dermatomyositis' because of normal serum CPK levels, normal EMG and no histological abnormality by muscle biopsy. Clinical manifestations improved by the treatment with corticosteroids. During tapering of corticosteroids, however, intersititial pneumonitis developed and rapidly progressed. The first patients was treated with methylprednisolone pulse therapy, azathiopurine and methotrexate. The second patients was treated with betamethazone, methlprednisolone pulse therapy and cyclosporin A. In spite of these extensive immunosuppressive therapies, both patients died of pulmonary insufficiency a few months after admission. In the literature there has been only several cases of amyopathic dermatomyositis and only one case with fatal rapidly progressive interstitial pneumonitis. A new approach to the treatment of this disease should be made.

Autoimmune Diseases↗

[Dermatomyositis as a paraneoplastic syndrome].

Course, diagnostics and therapy of paraneoplastic dermatomyositis are described by own observation. The coincidence of dermatomyositis and carcinomas provoke to look for a malignoma or to register beginning complications after primary treatment in time of a malignancy

Biopsy↗