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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Characteristics of juvenile dermatomyositis in Japan. 914 Dec 68

Calcinosis cutis in dermatomyositis is dystrophic calcification appearing late in the course of the disease. Two cases are reported here of calcinosis cutis that presented years before other clinical manifestations of juvenile dermatomyositis. The first case was a 14-year-old Thai girl who had asymptomatic subcutaneous nodules that spontaneously ruptured, exuding a chalky discharge and healing with an atrophic scar 8 years before the onset of other clinical manifestations of juvenile dermatomyositis; that is, Gottron's papules, proximal muscle weakness grade IV/V with atrophy, slightly elevated serum creatinine phosphokinase level and an abnormal electromyogram compatible with myopathy. The second case was a 15-year-old Thai boy who had calcinosis cutis 3 years before the onset of other clinical manifestations of juvenile dermatomyositis, and the calcinosis cutis was so severe that it interfered with the movement of his extremities. Both cases responded well to aluminium hydroxide therapy.
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PMID:Calcinosis cutis presenting years before other clinical manifestations of juvenile dermatomyositis: report of two cases. 943 16

Dermatomyositis (DM) is an idiopathic inflammatory myopathy associated with characteristic cutaneous and extracutaneous manifestations, including malignancy. Primary fallopian tube carcinoma (FTC) is the least common site of origin for a malignant neoplasm of the female genital tract. This report describes the first documented case of DM and concurrent FTC in the United States. A 62-year-old woman presented with DM and was subsequently found to have FTC. During her clinical course, she had improvement in strength and function with treatment of the underlying FTC, which also correlated with lower creatinine phosphokinase levels. An association between DM and FTC may exist because of significant functional strength improvements after tumor removal and chemotherapy.
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PMID:Dermatomyositis associated with fallopian tube carcinoma. 986 7

Interstitial pneumonitis is a severe complication of juvenile dermatomyositis. We report a 4-year-old girl with juvenile dermatomyositis. Coughing, shortness of breath, and general malaise developed during steroid treatment. The histology of her lung biopsy is compatible with interstitial pneumonitis. Aggressive treatment including intravenous methylprednisolone pulse therapy, intravenous immunoglobulin, and oral cyclosporin all failed. Creatinine phosphokinase level was within the normal range during the disease course. The clinical features are discussed and the importance of a differential diagnosis of interstitial pneumonitis in patients with juvenile dermatomyositis is emphasized.
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PMID:Steroid refractory interstitial pneumonitis in a patient with juvenile dermatomyositis. 1254 53

We present 3 patients with juvenile dermatomyositis (JDM) and severe central nervous system (CNS) complications. All patients had at least 4 positive criteria of Bohan and Peter, which confirmed a definite diagnosis of JDM. They were all male, and had a relatively high creatinine kinase value at admission (1532-4260 U/l). Besides, progressive proximal muscle weakness and rash, one patient presented with rapid irreversible decline of vision. Ophthalmologic examination showed active vasculitis of the retina. After 2 weeks of treatment with immunosuppressive drugs and being in improved, relatively stable clinical condition, all 3 patients developed generalized tonic-clonic convulsions. Other causes of the neurological symptoms could be excluded. In all 3 patients, the course of JDM was fatal. The clinical symptoms and further investigations in our patients show CNS involvement in JDM. Although rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JDM.
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PMID:Case report: severe central nervous system involvement in juvenile dermatomyositis. 1296 16

Transient weakness of the legs developed in a 17 year-old male high school student three weeks after the initial injection of a hepatitis B vaccine. Seventeen days after the second injection of the vaccine, low-grade fever, a pruritic maculopapular rash appeared and weakness of the legs recurred. This was associated with elevation of the creatinine kinase to 2,406 U/L. The day after admission he became afebrile and in the subsequent four days the rash improved but leg weakness persisted. One-month later, muscle strength had returned; and the creatinine kinase had returned to normal levels. The only case of dermatomyositis associated with hepatitis B vaccination and the findings in the six reported cases of surface antigen-positive hepatitis associated with polyomyositis or dermatomyositis are briefly reviewed. Hepatitis B vaccination should be encouraged, but it is important to be aware that, rarely, dermatomyositis, polymyositis or neurovascular complications may occur. Polymyositis associated with the administration of the hepatitis B vaccine or with hepatitis B virus infection is a rare occurrence. A Medline Search performed from 1960 to January 2002 associating hepatitis B vaccine or hepatitis B virus with myopathy, myositis, polymyositis and dermatomyositis, showed only one case of dermatomyositis related to the hepatitis B vaccine, and six case reports relating polymyositis to hepatitis B virus infection. We present a case where a causal relationship between polymyositis and hepatitis B vaccination appears quite likely.
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PMID:Polymyositis: rare complication of hepatitis B vaccination. An unusual cause of toxic shock syndrome. 1544 86

A 40-year-old Chinese-American woman with breast carcinoma metastatic to her lungs presented with a 3-month history of erosions on her inner thighs (Figure 1) and medial left shoulder. Faint livedo reticularis was evident on her legs as well. She had difficulty in walking and raising her shoulders. Her cutaneous examination was also notable for cuticular erythema (Figure 2) and mild periorbital erythema and edema. She had no systemic or rheumatologic complaints other than some difficulty in swallowing. Her blood chemistry values were notable for a creatinine kinase of 564 IU/L (5-200 IU/L), alanine aminotransferase 161 U/L (0-40 U/L) and aspartate aminotransferase 93 U/L (0-40 U/L), and an antinuclear antibody titer of 1:2560. Other blood chemistries and antibody serologies (anti-Jo-1, anti-Mi-2 and other anti-tRNA synthetase, anti-Ro/SSA, anti-U1RNP, anti-PM/Scl, and anti-Ku) were within normal limits. A biopsy specimen was obtained from an area of intact skin close to a right thigh ulceration that showed subtle vacuolar alteration at the dermo-epidermal junction with occasional necrotic keratinocyte (Figure 3). Melanophages and telangiectases were present. Within the subcutis there was fibrin deposition and neutrophils. A diagnosis of dermatomyositis was made. The patient received oral prednisone 20 mg three times a day, and her ulcerations resolved. Her creatinine kinase, alanine aminotransferase, and aspartate aminotransferase values returned to normal over the course of 3 weeks, but her antinuclear antibody was unchanged. Radiographic studies concurrently noted that her breast cancer had recurred in her lungs; plans were made to treat her with chemotherapy. The patient was lost to close follow-up, but it was learned that her erosions had reoccurred while her prednisone was tapered and resolved when her dosage of prednisone was increased.
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PMID:Ulcerative paraneoplastic dermatomyositis secondary to metastatic breast cancer. 1660 44

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.
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PMID:Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis. 1746 14

It is interesting to study an autoimmune condition like dermatomyositis (DM) in the setting of immunosuppression due to human immunodeficiency virus (HIV) infection. An HIV seropositive female aged 30 years, presented with a nonitchy rash over the face, breathlessness, diarrhoea and difficulty in raising her hands above her head. A heliotrope rash around the eyes, Gottron's papules and proximal muscle weakness were found to be present. C reactive protein, erythrocyte sedimentation rate and lactate dehydrogenase levels were raised, but creatinine phosphokinase and anti-nuclear antibody profile were normal. Her HIV serostatus was confirmed by Western blotting, keeping in mind the potential for false positive HIV serology in an autoimmune disorder. Her CD4 count was 379 cells/mm3. An X-ray of the chest showed bilateral pleural effusion with raised pleural fluid adenosine deaminase levels. Clinical findings and laboratory investigations favored the diagnosis of DM and HIV infection with tuberculous effusion in an HIV seropositive patient. She was treated with antibiotics, four-drug anti-tubercular treatment, systemic steroids and later, antiretroviral treatment. Chances of a false positive antibody test for HIV should be considered in a patient having an autoimmune disease such as DM.
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PMID:Dermatomyositis in a human immunodeficiency virus infected person. 1858 92

A 66-year-old man was admitted for progressive proximal weakness and myalgia in his shoulder girdles without skin lesions. A muscle biopsy showed infiltration of inflammatory cells, degeneration of muscle fibers, and perifascicular atrophy. Remarkably, MHC-I antigen was expressed in the muscle membrane and most of the inflammatory cells were CD8-positive, suggesting that class-I antigen-dependent cytotoxic T-cells played a crucial role in the muscle pathology, which supported a diagnosis of polymyositis rather than dermatomyositis. Magnetic resonance imaging of his chest revealed two mediastinal tumors, which were extirpated and diagnosed as thymic carcinoma. The muscle weakness was completely recovered after the operation and subsequent administration of oral prednisolone. Postoperative clinical improvement and decline of serum creatinine kinase suggested a paraneoplastic nature of the polymyositis in this patient.
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PMID:Polymyositis with atypical pathological features associated with thymic carcinoma. 1918 27


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