Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report two cases of the Shulman's syndrome. Twenty one cases of this new entity are found in the literature. The main signs are the acute painful onset after an unusual physical exertion, the development of fasciitis, and (or) myositis, and (or) scleroderma of limbs, the absence of Raynaud's phenomenon, visceral involvement, constant eosinophilia and hypergammaglobulinemia; the prognosis is good with an improvement sometimes spontaneous or coincident with prednisone therapy. One of the observations is interesting by the association with Gougerot-Sjogren's syndrome, and a familial case of morphea. The prognosis in this case is mediocre: failure of corticotherapy, and incomplete remission with cyclophosphamide.
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PMID:[Shulman's syndrome: fasciitis with eosinophilia, pseudoscleroderma with eosinophilia]. 61 May 15

The term fasciitis-panniculitis syndrome (FPS) is proposed as a novel compilation encompassing several disorders, common to which is subcutaneous induration caused by cicatrizing fasciitis as well as septal and lobular panniculitis and perimysial fibrosis. Included herein are Shulman's eosinophilic fasciitis, morphea profunda, lupus profundus, venous lipodermatosclerosis, toxic oil syndrome, altered tryptophane-related eosinophilic myositis, graft-versus-host reaction, and fasciitis reactive to subjacent basal cell carcinoma. FPS should be differentiated from scleroderma, which primarily affects the dermal structures and in which arterioles are injured. In contrast, vasculopathy of the subcutaneous medium-sized veins accompanies the hypodermal lesions of FPS. The importance of recognizing and grouping these disorders lies in their different histopathology, characterization as reactive phenomena, enhanced responsiveness to treatment, and better prognosis than scleroderma. In view of the excellent prognosis of FPS, steroid treatment is not warranted. Long-term therapy with cimetidine appears to benefit the majority of patients.
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PMID:The fasciitis-panniculitis syndrome: clinical spectrum and response to cimetidine. 157 May 16

In 44 patients with morphea the incidence of internal organ involvement was studied. For the clinical study, only patients with disseminated (22 patients), linear (20 patients), and generalized morphea (2 patients) were considered. Systemic parameters were determined for inflammation and the function of the esophagus, lung, heart and kidneys. In 22 patients, mostly with the linear form of morphea, the muscles were studied by electromyography. In 23 patients the HLA-A, HLA-B, HLA-C and HLA-DR patterns were determined; 27% of the patients showed systemic organ manifestations. Esophagus function was impaired in 10 and lung function in 6 cases; 15 patients showed myositis. The degree of systemic involvement was correlated with the type of morphea and the grade of systemic inflammation. Generalized morphea showed a high rate of organ involvement (2 of 2); in linear morphea organ involvement was reduced to 34% and in the disseminated form, to 14%. There was a significant association of HLA-DR1 and -DR5 with the different types of morphea.
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PMID:[Circumscribed scleroderma: internal manifestations and significant correlation to HLA-DR1 and DR5]. 387 10

The most common scleroderma overlap syndromes are mixed connective tissue disease (MCTD), scleromyositis and synthetase syndrome. There is controversy concerning MCTD as a separate entity due to heterogeneous clinical manifestations, not infrequent transformation into definite CTD and various classification criteria. Our study of 94 adult patients and 20 children, classified according to the criteria of Alarcon-Segovia, and especially a 5, 9-year follow-up showed transformation into SLE or SSc in over 20% of patients, less frequently than reported by others, whereas over half of the cases remained undifferentiated CTD. In several cases ARA criteria for both SSc and SLE were fulfilled, and there is no consensus whether such cases should be recognized as coexistence of both definite diseases or as MCTD. High titers of U1 RNP antibodies to 70 kD epitope were invariably present, whereas, by transformation into distinctive CTD there appeared, in addition, antibodies characteristic of these CTD. Of 108 cases positive for PM-Scl antibody, 83% were associated with scleromyositis. This scleroderma overlap syndrome differed from MCTD by coexistent features of dermatomyositis (myalgia, myositis, Gottron sign, heliotrope rash, calcinosis) with no component of SLE, characteristic of MCTD. The course was also chronic and rather benign, as in MCTD, and all cases responded to low or moderate doses of corticosteroids. A not infrequent complication was deforming arthritis of the hands. Our immunogenetic study showed an association of cases positive for PM-Scl antibody with HLA-DQA1x0501 alleles in 100% and with HLA-DRB1x0301 in 94% of cases. Synthetase syndrome, associated with anti-histidyl-tRNA synthetase antibodies, studied in 29 patients with myositis and interstitial lung disease (ILD), only in single cases had scleroderma-like features. These cases differed from SSc by acute onset with fever, and by response to moderate doses of corticosteroids. We also studied overlap of localized scleroderma with other CTD: 21 cases of progressive facial hemiatrophy and linear scleroderma, and 55 (39.5%) of atrophoderma Pasini-Pierini (APP) and morphea. As in other autoimmune disorders, two or more connective tissue diseases (CTD) may develop concurrently or sequentially in the same patient. In such overlap syndromes ARA criteria must be fulfilled for each of the disease, and the clinical presentation has features of both. However more frequently overlap syndromes only combine some manifestations of more than one CTD, and present a highly heterogeneous group of disorders with prevailing clinical features of SSc.
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PMID:Scleroderma overlap syndromes. 1059 27

Localized scleroderma is characterized by idiopathic fibrosis of the skin and adjacent structures, and muscle involvement occurs predominantly in deep morphea. We report a patient with linear scleroderma who presented with slowly progressive atrophy, muscle weakness, and loss of function of her right arm, mimicking a neuromuscular disorder. Muscle biopsy eventually revealed zones of myositis, compatible with morphea profunda. Morphea profunda may thus present as a neuromuscular mimic, even in case of nonprogressive skin sclerosis. Myositis in morphea profunda is generally limited to one region, whereas inflammatory myopathies generally cause diffuse proximal muscle weakness and atrophy. Furthermore, skin changes in morphea profunda differ from those seen in dermatomyositis, and histological features of muscle biopsy can further distinguish between morphea profunda and inflammatory myopathies. Muscle biopsy in morphea profunda implies the risk of sampling error, whereas results of electromyography and muscle imaging might better represent the extent of muscle inflammation.
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PMID:Morphea profunda presenting as a neuromuscular mimic. 1852 25

We report a 16-year-old Japanese girl with anti-Ku antibody-positive generalized morphea and polymyositis who, at the age of 7 years, developed multiple brownish plaques on her left forearm that gradually extended to her upper arm, back, and left thigh, which a skin biopsy revealed as morphea. Laboratory testing was positive for antinuclear antibody and a high serum creatine kinase level. Although there were no clinical signs of muscular involvement, magnetic resonance imaging revealed findings consistent with myositis. The patient is one of the youngest reported cases positive for anti-Ku antibody. Anti-Ku positivity concomitant with generalized morphea is rare.
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PMID:Childhood-Onset Anti-Ku Antibody-Positive Generalized Morphea with Polymyositis: A Japanese Case Study. 2620 36

Morphea is an autoimmune disorder characterized by sclerosis and inflammation of the skin and soft tissues. Early diagnosis and treatment are essential to minimize morbidity such as joint contracture. In this report, we present the case of a 19-year-old man with linear morphea with inflammatory myositis who presented to our clinic 1 year after symptom onset with severe elbow flexion contracture. Through reviewing this rare disorder, it is hoped that early diagnosis will lead to better outcomes in the future.
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PMID:Linear Morphea With Inflammatory Myositis. 3180 51