1/62. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis.We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases.- - - - - - - - - - ranking = 1keywords = myositis (Clic here for more details about this article) |
2/62. scleromyxedema.scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis. The patient's serum contained Scl 70 antibodies, characteristic of scleroderma. electromyography showed signs of acute myositis and the creatine phosphokinase (CPK) level was elevated. Multiply passaged fibroblasts from the patient's skin lesions showed altered growth response in vitro. The patient was treated with cyclosporin (4 mg/kg/day) with improvement.- - - - - - - - - - ranking = 0.33333333333333keywords = myositis (Clic here for more details about this article) |
3/62. Localized subcutaneous nocardia farcinica abscess in a woman with overlap syndrome between systemic scleroderma and polymyositis.nocardia farcinica is an increasingly common cause of human infection in europe. We observed an isolated nodular subcutaneous abscess due to N. farcinica in the forearm of a young patient with overlap syndrome between systemic sclerosis and polymyositis. Despite in vitro resistance to trimethoprim-sulfamethoxazole, this oral combination completely resolved the lesion within 3 weeks and was maintained for 6 months. The spectrum of cutaneous nocardiosis in the immunocompromised host is discussed.- - - - - - - - - - ranking = 11.249923799701keywords = polymyositis, myositis (Clic here for more details about this article) |
4/62. mixed connective tissue disease.Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.- - - - - - - - - - ranking = 0.16666666666667keywords = myositis (Clic here for more details about this article) |
5/62. A juvenile case of overlap syndrome of systemic lupus erythematosus and polymyositis, later accompanied by systemic sclerosis with the development of anti-Scl 70 and anti-Ku antibodies.We describe a 16-year-old girl with an overlap syndrome consisting of systemic lupus erythematosus (SLE) from the age of 7 and polymyositis (PM) from the age of 10, later accompanied by systemic sclerosis (SSc) from the age of 15. She was diagnosed as having SLE with exudative malar erythema, photosensitivity, and discoid rashes with positive antinuclear antibody (ANA) and anti-dna antibody titers. The diagnosis of PM was also made in accordance with findings of a high titer of muscle enzymes and a muscle biopsy specimen demonstrating marked degeneration of the muscle fibers and perivascular infiltration of mononuclear cells. She developed Raynaud's phenomenon and pitting ulcers on her fingers with positive anti-Scl 70 and anti-Ku antibodies, leading to a diagnosis of SSc. The patient was treated with prednisolone. To our knowledge this is the youngest case of SLE-PM overlap syndrome later accompanied by SSc.- - - - - - - - - - ranking = 11.249923799701keywords = polymyositis, myositis (Clic here for more details about this article) |
6/62. Myositis and interstitial lung disease associated with autoantibody to a transfer rna-related protein Wa.We describe 2 patients with myositis and interstitial lung disease with the autoantibody to Wa antigen, a 48-kDa transfer rna-related protein. In contrast to the previous description of anti-Wa antibody, our patients lacked systemic sclerosis-related features, but had clinical features consistent with those associated with antibodies to aminoacyl-transfer rna synthetases. The difference in clinical presentation between our patients and patients with systemic sclerosis may be explained by recognition of different epitopes on the Wa antigen.- - - - - - - - - - ranking = 0.16666666666667keywords = myositis (Clic here for more details about this article) |
7/62. Progressive systemic sclerosis (scleroderma), carcinoma breast and valvular heart disease: an unusual combination.Systemic sclerosis is a multi system disorder characterised by fibrosis of skin and internal organs. There are reports of relation between cancer and polymyositis/dermatomyositis, but no overall association with systemic sclerosis. Reports of the coexistence of cancer and systemic sclerosis, however, emphasise a close temporal relation in their occurrence. Cardiac involvement in the form of myocardial fibrosis and pericarditis occurs frequently in systemic sclerosis, while valvular involvement has been reported only sporadically. We report a patient, admitted for adenocarcinoma of left breast who was found to have features of systemic sclerosis, pulmonary hypertension, gangrene of toes, and stenotic mitral valve disease. The possible mechanisms of the coincidence of the three disorders are discussed.- - - - - - - - - - ranking = 2.4166514266069keywords = polymyositis, myositis (Clic here for more details about this article) |
8/62. Anti-Ku antibody-positive scleroderma-dermatomyositis overlap syndrome developing Graves' disease and immune thrombocytopenic purpura.Graves' disease (GD) has been reported to be frequently complicated with other autoimmune diseases. However, it is rarely complicated with scleroderma-polymyositis overlap syndrome. Recently, we encountered a 35-year-old woman who developed GD and immune thrombocytopenic purpura during follow-up observation of scleroderma-dermatomyositis overlap syndrome. Platelet counts recovered after high-dose gamma-globulin therapy and bolus methylprednisolone therapy. The present case is the first report of a combination of scleroderma, dermatomyositis, GD, and immune thrombocytopenic purpura. The patient was anti-Ku antibody-positive and had relatively low natural killer T cell counts, both of which might contribute to the complication of multiple autoimmune diseases.- - - - - - - - - - ranking = 3.2499847599403keywords = polymyositis, myositis (Clic here for more details about this article) |
9/62. Two sisters producing anti-U1RNP exhibit serological concordance and clinical discordance.Two sisters had autoimmune responses to the U1RNP particle that were quantitatively similar and/or identical in molecular characteristics. No other autoantibodies were demonstrable. Both sisters immunoprecipitated only U1RNA, had a reaction of identity in gel diffusion, bound the 68-kDa band in HeLa cell extract in Western blot, and reacted almost equally to a rabbit anti-idiotypic reagent made against either sister's isolated anti-U1RNP Fab fragments. They both carried a DR4 allele, which has been associated with anti-U1RNP production in several studies. While the sisters both had Raynaud's phenomenon, their clinical pictures were otherwise dissimilar. One had a seizure disorder (Ju); the other had polymyositis and features of scleroderma (Je). In sister Ju, Raynaud's phenomenon was manifest for the first time in association with the appearance of precipitating anti-U1RNP.- - - - - - - - - - ranking = 2.2499847599403keywords = polymyositis, myositis (Clic here for more details about this article) |
10/62. neurologic manifestations of progressive systemic sclerosis.Neurologic involvement in progressive systemic sclerosis is considered uncommon. We retrospectively examined the prevalence and nature of neurologic complications in 50 patients with progressive systemic sclerosis. In 20 (40%), neurologic abnormalities were detected, with a total of 28 neurologic manifestations. All levels of the central and peripheral nervous system were affected: muscle (22%), peripheral nerve (18%), spinal cord (8%), and brain (6%). Of note were the presence of myelopathy in four patients and inclusion-body myositis in two. In 10 patients (20%), no definable cause of the neurologic dysfunction could be identified, apart from progressive systemic sclerosis. Thus, neurologic presentations of progressive systemic sclerosis are much more common than previously reported and may be due to direct involvement of the nervous system by a primary pathologic process in a significant number of patients.- - - - - - - - - - ranking = 0.16666666666667keywords = myositis (Clic here for more details about this article) |
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