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

Three patients are described with clinical features of connective tissue diseases, namely, dermatomyositis, progressive systemic scleroderma, and systemic lupus erythematosus. In two patients the symptoms of disease overlapped. The rare anti-Ku antibody was found in all of them. Anti-Ku antibody characterizes those patients, with polymyositis-scleroderma overlap syndromes who have a good prognosis. One of our patients, who also had severe anti-Sm-positive systemic lupus erythematosus, seems to be an exception.
J Am Acad Dermatol 1989 Aug
PMID:Anti-Ku antibodies in connective tissue diseases. Report of three cases. 278 12

Dermatomyositis/polymyositis are multisystem disorders affecting both children and adults. The issues regarding malignancy are still unresolved. Is the patient with DM more prone to malignancy than the patient with PM? Is malignancy actually related to the myositis in the meaningful fashion? How should the patient with myositis be evaluated for a potential malignancy neoplasm? The disease is a truly multisystem disease that involves the joints, lungs, heart muscle, and the gastrointestinal system. Newer serologic tests are of benefit in understanding the pathogenesis of the disease and are currently of little use to the clinician. Finally, the therapy for DM can be difficult. Antimalarial use in cutaneous disease can be used for patients who are steroid resistant or who develop steroid side effects. Multiple approaches have been developed but await confirmation regarding their effectiveness in future studies.
Adv Dermatol 1988
PMID:Dermatomyositis: current controversies 1987. 315 26

We performed thallium 201 muscle scans to assess muscular involvement in 40 patients with different connective tissue diseases (7 with dermatomyositis, 7 with systemic lupus erythematosus, 12 with progressive systemic scleroderma, 2 with calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia (CREST) syndrome, 3 with monomelic scleroderma, 6 with morphea, and 3 with Raynaud's disease). Only 12 of these patients complained of fatigability and/or myalgia. Electromyography was performed and serum levels of muscle enzymes were measured in all patients. Comparison of thallium 201 exercise recording with the other tests revealed that scan sensitivity is greater than electromyographic and serum muscle enzymes levels. Thallium 201 scans showed abnormal findings in 32 patients and revealed subclinical lesions in 18 patients, while electromyography findings were abnormal in 25 of these 32 patients. Serum enzyme levels were raised in only 8 patients. Thallium 201 scanning proved to be a useful guide for modifying therapy when laboratory data were conflicting. It was useful to evaluate treatment efficacy. Because our data indicate a 100% positive predictive value, we believe that thallium 201 scanning should be advised for severe systemic connective tissue diseases with discordant test results.
J Am Acad Dermatol 1988 Apr
PMID:Evaluation of muscular lesions in connective tissue diseases: thallium 201 muscular scans. 337 58

Vitamin K-dependent carboxylase is demonstrated in skin microsomes from humans, rats, rabbits, and mice. This enzyme converts a number of distinct protein-bound glutamic acid residues into gamma-carboxyglutamic acid residues, which strongly interact with Ca++ ions. The enzymatic activity (expressed per mg protein) in skin is about 20% of that in liver. Vitamin K-dependent carboxylase is present in both epidermal and dermal tissue. It is demonstrated that warfarin treatment in mice results in an accumulation of noncarboxylated precursor proteins in both dermal and epidermal microsomes. Most probably this effect of warfarin is not restricted to mice, but occurs also in the skin of patients under oral anticoagulant therapy. A possible relation between vitamin K-dependent skin carboxylase and the gamma-carboxyglutamic acid-containing protein in calcified nodules from patients with scleroderma and dermatomyositis is discussed.
J Invest Dermatol 1986 Sep
PMID:Vitamin K-dependent carboxylase in skin. 348 54

Out of 97 patients with circulating ribonucleoprotein antibodies, 44 (45%) satisfied the criteria for systemic sclerosis, systemic lupus erythematosus, polymyositis/dermatomyositis, or rheumatoid arthritis. Forty-two (43%) of the 97 patients whose cases did not fulfill these criteria had at least two of the following three clinical manifestations: arthritis, Raynaud's phenomenon, and swollen or sclerotic fingers. A fifth of the latter group of patients had chronic, restrictive pulmonary disease or myopathy and two thirds had hypergammaglobulinemia, IgM rheumatoid factor, and sensitized epidermal nuclei. Few patients had hypocomplementemia. One patient had nephropathy. Most patients had an unchanged, benign disease course for, on the average, nine years. It is suggested that the term mixed connective tissue disease (MCTD) be reserved for such patients, and that the acronym MCTD be changed to SRA (swollen fingers, Raynaud's phenomenon, and arthritis). Treatment with glucocorticoids is necessary for only a minority of patients.
Arch Dermatol 1987 May
PMID:Clinical implications of ribonucleoprotein antibody. 349 41

Although fibrosing alveolitis is a rare complication of dermatomyositis, early detection and treatment are important in preventing pulmonary fibrosis. Anti-Jo-1 antibody, an antibody to the cellular enzyme histidyl-t-RNA synthetase, has been found to correlate closely with the subset of dermatomyositis/polymyositis associated with fibrosing alveolitis. This association is well known to rheumatologists but has received little attention in the dermatologic literature. We wish to alert dermatologists to the importance of the association of anti-Jo-1 antibody in patients with dermatomyositis and present a patient with anti-Jo-1 antibody who had Raynaud's phenomenon and mild arthritis in association with dermatomyositis and pulmonary fibrosis. We suggest screening patients with polymyositis/dermatomyositis for this antibody, to detect the population at high risk of developing pulmonary complications.
J Am Acad Dermatol 1987 Aug
PMID:Dermatomyositis and pulmonary fibrosis associated with anti-Jo-1 antibody. 349 58

Diseases in which involvement of the nails may be helpful in the diagnosis of dermatologic disease elsewhere include psoriasis, lichen planus, Darier's disease, alopecia areata and totalis, keratotic scabies, scleroderma, and lupus erythematosus and dermatomyositis. Dermatologic diseases in which involvement of the nail commonly occurs but is relatively nonspecific and not diagnostic include dermatitis, lichen striatus, parakeratosis pustulosa, pityriasis rubra pilaris, acrokeratosis paraneoplastica, pemphigus vulgaris, epidermolysis bullosa, porokeratosis of Mibelli, and acanthosis nigricans. These entities are described and treatments summarized as appropriate.
Dermatol Clin 1985 Jul
PMID:Dermatologic diseases of the nail unit other than psoriasis and lichen planus. 383 May 3

We investigated 24 juvenile cases of linear scleroderma for the presence of systemic disease and serologic abnormalities. Thirteen of 24 patients had antinuclear antibodies (ANA) at titers of 1:40 or greater. Rheumatoid factor (titers greater than or equal to 1:20) was detected in seven of 17 patients tested, five of whom also had ANA. Two of five patients with ANA and rheumatoid factor had systemic diseases, such as nephritis and Raynaud's phenomenon. One patient with ANA developed typical dermatomyositis. Consequently, patients with linear scleroderma may be at some risk for developing systemic collagen-vascular diseases. On initial presentation, patients with linear scleroderma should give a complete history and receive a thorough physical examination as well as undergo laboratory evaluations for the presence of ANA and rheumatoid factor. Long-term observation with periodic reevaluation is appropriate for many members of this group.
Arch Dermatol 1985 Nov
PMID:Juvenile linear scleroderma associated with serologic abnormalities. 387 8

Epidermal nuclear deposits of immunoglobulins (Ig) were studied by direct immunofluorescence in three groups of patients: ten scleroderma (SD, systemic sclerosis), seven dermatomyositis (DM) and seven systemic lupus erythematosus (SLE). Each patient had skin biopsies taken from three different sites (nailfold, forearm, buttock) on the same day that a serum sample was also obtained. Epidermal nuclear deposits were observed in nine of twenty-four patients (five SD, two DM, two SLE). A high serum ANA titre correlated significantly with the presence of epidermal nuclear Ig deposits. The nucleolar epidermal nuclear pattern was limited to the SD group, four of ten patients showing this pattern. Two of nine patients with positive results in the nailfold and forearm had negative findings in the buttock, supporting the view that deposition of Ig in the epidermal nuclei occurs in vivo.
Br J Dermatol 1985 Jan
PMID:Epidermal nuclear immunofluorescence: serological correlations supporting an in vivo reaction. 388 23

The classic features of childhood dermatomyositis include muscle weakness, elevated muscle enzymes, and characteristic abnormal muscle biopsy and electromyography. Paramount to the diagnosis are cutaneous dermatoses that include a heliotrope rash and Gottron's papules. Rarely, a photo-sensitive dermatosis may occur. A recurrent photoexacerbated dermatoses can be an initial sign of occult childhood dermatomyositis.
Pediatr Dermatol 1985 Mar
PMID:Recurrent photosensitive dermatitis preceding juvenile dermatomyositis. 399 76


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>