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)

A 60-year-old woman was admitted to our hospital with complaints of muscle weakness and erythema on her extremities. Gottron's sign, heliotrope rash, elevation of serum myogenic enzymes, electromyography and magnetic resonance imaging findings established a diagnosis of dermatomyositis (DM). She was treated with 60 mg of daily prednisolone. One week later, she suddenly developed splenic and renal infarctions, which were considered to have resulted from vasculopathy associated with DM. Cyclophosphamide and anticoagulants along with increasing the dosage of corticosteroid were effective. This is the first report describing splenic and renal infarctions in a patient with adult-onset DM.
 ...
PMID:Dermatomyositis with splenic and renal infarctions during corticosteroid therapy. 1085 60

Although sarcoidosis has occasionally been associated with hemoglobinopathies, its association with dermatomyositis is extremely rare. A 24 y/o African American male with hemoglobin SC disease developed proximal weakness. He had heliotrope rash, muscle weakness, elevated serum CK, myositis by EMG, and no malignancy. A muscle biopsy confirmed dermatomyositis. Two years later, he was hospitalized with constitutional and respiratory symptoms and parotid enlargement. Chest X-ray and CT scan showed diffuse micronodular infiltration in both lungs and mediastinal lymphadenopathy. A transbronchial lung biopsy revealed non-caseating granulomata consistent with sarcoidosis. AFB and fungal stains and cultures were negative. Thus, in this case, sarcoidosis developed after or in association with dermatomyositis. A review of world's literature showed five other non-identical cases of dermatomyositis associated with sarcoidosis.
 ...
PMID:Sarcoidosis and dermatomyositis in a patient with hemoglobin SC. A case report and literature review. 1095 67

'Amyotrophic dermatomyositis' (ADM) is used to describe a small subgroup of patients with the typical skin rash associated with dermatomyositis but without muscle involvement. Lung involvement in ADM is rare. We report on the management of a patient with ADM associated with pulmonary fibrosis at presentation, and her response to corticosteroid treatment.
 ...
PMID:Amyopathic dermatomyositis and pulmonary fibrosis. 1114 62

Dermatomyositis (DM) is an inflammatory myopathy characterized by a typical cutaneous rash and proximal weakness. DM results from endothelium deposition of the complement membranolytic attack complex C5b-9, followed by inflammation. It is thus possible to have cases of DM with the typical rash, but with no associated myositis--amiopathic DM. DM represents a higher risk of association with malignancy and sometimes behaves as a paraneoplastic syndrome. As DM can be the alarm sign, once diagnosed, exclusion of malignancy should be done. In this article we also discuss the treatment and prognosis of dermatomyositis, according to the literature.
 ...
PMID:[Classic/amyopathic dermatomyositis]. 1123 94

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.
 ...
PMID:Scleromyxedema (lichen myxedematosus) associated with dermatomyositis. 1125 73

A 59-year-old man was admitted presenting systemic rash and muscle weakness. He was diagnosed to have dermatomyositis and a check was made for internal malignancy. Gastrointestinal endoscopy revealed a Borrmann type II tumor on the middle body of the stomach. Biopsy specimens showed a well differentiated adenocarcinoma, and total gastrectomy was performed. The final diagnosis was moderately differentiated adenocarcinoma invading into the proper muscular layer, with metastases to regional lymph nodes. Most of the neoplastic cells were shown to be positive for Epstein-Barr virus by means of EBV-encoded RNA in situ hybridization. The symptoms of dermatomyositis disappeared completely after surgery.
 ...
PMID:Epstein-Barr virus-associated gastric cancer in a patient with dermatomyositis. 1130 Jan 69

Malar rash and photosensitivity are common findings in systemic lupus erythematosus (SLE). However, a number of inflammatory, dermatologic, infectious or drug-induced conditions may mimic cutaneous findings of SLE. These typically include seborrheic dermatitis, contact dermatitis, rosacea, polymorphous light eruption, syphilis and dermatomyositis sine myositis. Herein we describe a patient with fever, malar rash, alopecia, photosensitivity, arthralgias and lymphadenopathy who was subsequently diagnosed with secondary syphilis. In this case report we review clinical and histopathological findings in the differential diagnosis of malar rash and photosensitivity and discuss the overlapping features of syphilis and SLE.
 ...
PMID:Lues and lupus: syphilis mimicking systemic lupus erythematosus (SLE). 1134 Nov 8

Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.
 ...
PMID:Amyopathic dermatomyositis. 1147 54

Little is known concerning factors associated with the outcome of juvenile dermatomyositis (JDM), which can be variable and lethal. Previous work has documented that the association of DQA1*0501 with JDM is higher than in control groups and that the first symptoms (rash and weakness) of JDM appear to follow evidence of an infectious process--most frequently upper respiratory in nature. Preliminary data show that a long period of symptoms being left untreated before starting therapy and the TNF alpha-308A allele are associated with prolonged JDM symptoms requiring > or = 36 months of immunosuppressive therapy. A short duration of untreated disease is associated with a relative increase in CD8(+) T cells and CD56(+) natural killer (NK) cells in the untreated JDM muscle biopsy compared with a longer duration of untreated disease. The TNF alpha-308A allele is overrepresented in white children with JDM. In addition, it is associated with pathologic calcifications, increased production of TNF alpha by peripheral blood mononuclear cells in vitro and JDM muscle fibers in vivo, and occlusion of capillaries, which may be mediated in part by elevated circulating levels of thrombospondin-1, a potent anti-angiogenic factor. We speculate that DQA1*0501 is associated with JDM susceptibility to an infectious process, eliciting and activating NK cells early in the disease course. We conclude that the TNF alpha-308A allele indicates directly (or is a surrogate marker of) children with JDM who produce higher concentrations of TNF alpha in response to this undefined inflammatory stimulus, as well as increased concentrations of TSP-1 with resultant small vessel occlusion, contributing to subsequent disease chronicity.
 ...
PMID:Juvenile dermatomyositis: the association of the TNF alpha-308A allele and disease chronicity. 1156 68

Juvenile dermatomyositis is a rare, chronic multisystemic inflammatory disorder of unknown etiology, characterized by a typical skin rash and proximal muscle weakness. A retrospective study from the medical records of patients diagnosed as juvenile dermatomyositis was performed at Queen Sirikit National Institute of Child Health from 1988 to 1998. There were seven cases of juvenile dermatomyositis diagnosed according to the criteria of Bohan and Peter. Six cases were female and one case was male. The age of diagnosis ranged from 2.5 years to 11 years. (mean age was 7 +/- 3.6 years). The presenting symptoms were muscle weakness (6 cases), muscle pain (2 cases) and skin rashes (4 cases). All of the patients developed proximal muscle weakness of the lower extremities varying from grade 3 to grade 4. The cutaneous manifestations were heliotrope signs (6 cases), gottron's papules (2 cases), photosensitivity (2 cases) and calcinosis cutis (4 cases). Electromyography (EMG) was performed in 6 cases and revealed typical change of myopathic type. Elevated muscle enzymes were noted in all cases. Muscle biopsy was performed in 6 cases and was compatible with myositis. Oral prednisolone (1-2 mg/kg/day) was given in 6 cases and the muscle weakness improved. There was no mortality in this study. Four cases developed calcinosis cutis 1 to 3 years after muscle weakness and did not respond to any treatment. In conclusion, juvenile dermatomyositis is a disease which causes chronic disability in children. Early diagnosis and treatment can prevent morbidity and mortality. Calcification at the skin usually occurs after the onset of muscle weakness several months to years after diagnosis.
 ...
PMID:Juvenile dermatomyositis in Thai children. 1185 94


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