Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Described here is a 59 year old man with dermatomyositis and hypogammaglobulinemia. His muscle power improved after corticosteroid therapy, but extensive amyloidosis and repeated infections appeared. Bone marrow morphology suggested multiple myeloma, but treatment with cytotoxic drugs had no beneficial effect on the amyloidosis. Because of rapid progression of the amyloidosis and further infections, cytotoxic drug therapy was stopped, corticosteroid dosage was decreased, and supplementary immunoglobulin therapy was instituted. The infections occurred less frequently and the amyloidosis appeared to regress. This case suggests that immunosuppressive therapy may exacerbate amyloidosis. The literature is reviewed, and the possible role of humoral immunodeficiency in the pathogenesis of amyloidosis is discussed. It is suggested that supplementary immunoglobulin may be beneficial in amyloidosis.
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PMID:Amyloidosis associated with dermatomyositis and features of multiple myeloma. The progression of amyloidosis associated with corticosteroid and cytotoxic drug therapy. 5 87

A 67-year-old man developed dermatomyositis. Investigation revealed an oat cell carcinoma of the lung. When he developed unilateral gynaecomastia this tumour was found to secrete gonadotrophins. The gynaecomastia disappeared after treatment with an anti-oestrogen--tamoxifen. This use of tamoxifen has not previously been described. Partial tumour regression was obtained with several cytotoxic drug regimes. His dermatomyositis responded to this treatment and remains in remission.
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PMID:Gynaecomastia associated with gonadotrophin-secreting carcinoma of the lung. 19 79

A young man is reported with recurrent Staphylococcus aureus joint sepsis associated with dermatomyositis. His dermatomyositis failed to resolve on treatment with antimicrobial agents alone, indicating that if staphylococcal infection was the triggering event for the dermatomyositis then the subsequent process was apparently self perpetuating, requiring cytotoxic agents for its control. This case can be interpreted as possible further evidence for the triggering of autoimmune disease by infective agents.
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PMID:Dermatomyositis following chronic staphylococcal joint sepsis. 238 66

Juvenile dermatomyositis (JDMS) is a systemic vasculopathy characterized primarily by inflammation of skin and muscle. JDMS is identified in more than three per million persons per year, using established diagnostic criteria. Although originally thought to be a relatively homogeneous disease, new data confirm that heterogeneity in JDMS may be found at several levels and that each variant may be associated with a different disease course. Unlike adults with dermatomyositis, of whom more than 50% have a specific myositis-associated antibody (MSA), a much smaller number of children appear to test positive for a known MSA (about 10%), despite the evidence that more than 60% of children with JDMS test positive for antinuclear antibodies. In children, the most common MSA is directed against Mi-2, not toward one of the tRNA synthetases, such as tRNA histidine, as is found in 20% to 30% of adults with myositis. About 50% of children with JDMS have circulating evidence of endothelial cell damage (increased vWF:Ag), whereas others have different indicators of disease activity, such as elevated neopterin (> 60%) or increased circulating B cells with peripheral lymphopenia (> 80%). Newer modes of assessment of functional ability may help evaluate response to therapy. Finally, physicians with newly diagnosed (< 6 months) JDMS patients are urged to call the new National Institutes of Health Rare Disease Registry for New Onset Dermatomyositis (312-880-3333) to enroll their patients and for more information on the onset of this disease.
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PMID:An update on juvenile dermatomyositis. 851 18

Adult Still's disease is a febrile disorder of unknown etiology, characterized by spiking fever, arthralgia, leukocytosis and a typical rash. However, most of these clinical findings, except for the typical rash, are not specific to this disease: therefore, the typical rash is the most important clinical finding for diagnosing this disease and physicians often have difficulty in making a definite diagnosis without the typical rash. In our department and related institutions, we have encountered 10 patients with Adult Still's disease who fulfilled the preliminary criteria for classification as Adult Still's disease, proposed by the Adult Still's Research Committee in Japan. The three major criteria are fever, arthralgia and typical rash, and 8 of 10 patients had an atypical rash; one satisfied two major criteria, and had an atypical rash and the other satisfied three major criteria and had an atypical rash on her eyelids. Here, we present the two cases of Adult Still's disease with atypical rash. The first patient was a 36-year-old male with an itchy annular erythema chronicum migrans, frequently seen inpatients with Lyme's disease, on his back. His clinical symptoms improved and the erythema disappeared after treatment with corticosteroids. The second patient, a 17-year-old female, had three major findings. In addition to the typical rash on her face, she had a heliotrope rash, usually seen in patients with dermatomyositis, on her eyelids. The typical rash on her face was related to her other clinical manifestations, and improved after treatment with corticosteroids. However, the rash on her eyelids showed no improvement after steroid therapy, suggesting that the erythema was probably not related to Adult Still's disease. Typical rash in Adult Still's disease, defined as a macular or maculopapular nonpruritic salmon pink eruption, was demonstrated to have the highest relative value associated with relatively high sensitivity and specificity for the diagnosis of Adult Still's disease. However, we have to be aware that some patients with Adult Still's disease could also have an atypical rash.
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PMID:[Two cases of adult Still's disease with atypical rash]. 972 60

A 27-year-old male patient with calcinosis universalis resulting from dermatomyositis was successfully treated with low-dose warfarin. On his trunk and extremities, there were many subcutaneous calcified nodules, and knee flexion was difficult. After oral warfarin therapy for three years, the calcified nodules became smaller, and the knee mobility improved. His serum vitamin K level was abnormally high, decreased just after starting warfarin therapy, and then remained within the normal range. Since vitamin K has been known to play an important role in the Ca2+ binding process in bones or tissues, we suggest that this therapy is effective in reducing subcutaneous calcification through the vitamin K cycle.
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PMID:A case of calcinosis universalis successfully treated with low-dose warfarin. 986 84

To assess condition of the heart in polymyositis (PM) and dermatomyositis (DM) in terms of defects in the rhythm and conductivity, we carried out ECG, 24-hour ECG monitoring, EIA measurements of KPK MB-fraction and myoglobin, echocardiography and Doppler echocardiography. Maximal frequency of arrhythmia presenting as supraventricular and ventricular extrasystole occurred in patients with acute PM/DM. Blockade of the bundle of His left anterior branch was detected most frequently in patients with acute and subacute PM-DM and in those with high serum levels of KPK MB-faction and myoglobin. Frequency of rhythmic and conductivity disorders seems unrelated to the disease duration. We registered no alterations at rest either in intracardiac hemodynamics or myocardial contractility. The study of these parameters under exercise test may provide objective information on early defects in myocardial contractility.
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PMID:[Cardiac rhythm and conduction disorders in polymyositis and dermatomyositis]. 986 14

An 84-year old man was admitted to Mitoyo General Hospital because of progressive malaise and edematous erythema on both eyelids (Heliotrope erythema). He also noted blister on his neck as well as erythema on the extensor surface of finger joints (Gottron's sign), elbows and knees. Neither weakness nor pain of his proximal muscle was elicited on physical examination on admission. His blood test disclosed positive inflammatory signs (i.e., mild elevation of ESR and positive CRP) without elevated value of muscle enzymes. Electromyogram showed normal pattern. Infiltration of inflammatory cells was not revealed by histological examination of biopsied muscle. A diagnosis of 'amyopathic dermatomyositis' was made based on these observations. Computed tomography of his chest disclosed interstitial pneumonia spreading over both lower lung fields. Colon fiberscopy revealed a polyp in his descending colon, which was classified into group I on cytological examination. He was treated with two sets of methylprednisolone (mPSL) pulse therapy (500 mg/day, 3 consecutive days, intravenously) followed by 30 mg/day of oral prednisolone (PSL). His skin lesions responded well to the above treatment and the dose of oral PSL was tapered. One month after the initiation of treatment, severe stomatitis as well as a large ulcer beneath his tongue developed accompanying an intractable pain. Mucosal biopsy revealed necrotizing vasculitis in medium-sizedartery at the bottom of ulcer. Another set of mPSL pulse therapy brought a prompt relief of his symptom and prohibited the recurrence of oral lesion. It should be noted that our patient did not fulfill the diagnostic criteria for DM because of the lack of muscular symptoms whereas he had characteristic skin lesions. Regarding the frustration possibly encountered at the time of diagnosing amyopathic DM, both sensitivity and specificity of the skin lesion for the diagnosis of DM were investigated. Moreover, the rarity of blister as a skin manifestation of DM was discussed as well.
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PMID:[A case of amyopathic dermatomyositis presenting blister and oral ulcer]. 1069 7

We report a 23-year-old man suffering from an overlap syndrome of systemic scleroderma and dermatomyositis who died from severe dilated cardiomyopathy. Because his weakness involved predominantly muscles in the facio-scapulo-humeral regions, he was initially thought to have facioscapulohumeral muscular dystrophy (FSHD) at other hospitals. However, he had also Raynaud phenomenon and low voltages on electrocardiogram. His apparent facial weakness was mainly due to atrophic skin changes. Unlike FSHD, the deltoid and levator scapulae muscles were also atrophic. Deltoid muscle biopsy performed one year earlier at another hospital showed mild myopathic changes without inflammation, but there were scattered thick-walled endomysial capillaries, suggesting inflammatory myopathy. Biceps brachii muscle biopsy in our hospital showed marked inflammation with perifascicular atrophy. In this patient, the cardiac muscle involvement progressed together with the skeletal muscle inflammation before scleroderma became apparent.
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PMID:[A patient with dermatomyositis and systemic sclerosis with preferential facioscapulohumeral muscle involvement and fatal cardiomyopathy]. 1177 Nov 57

A 62-year-old man was admitted to hospital with general malaise, arthralgia, edema, and high-grade fever. He was diagnosed as dermatomyositis because of typical skin rashes and muscle weakness. His symptoms were improved by high-doses of prednisolone and cyclosporin A, with the exception of the skin rash over the back. High-grade fever developed again when tapering prednisolone. His condition deteriorated gradually. Paresthesia, hypothyroidism, metabolic acidosis, and disseminated intravascular coagulopathy occurred, and he died eight months after the first admission. Postmortem examination revealed intravascular proliferation of atypical mononuclear cells in the lumens of small vessels in all organs. Intravascular lymphomatosis (B cell type) was diagnosed.
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PMID:An autopsy case of intravascular lymphomatosis with dermatomyositis. 1192 90


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