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)

Deposition of calcium salts in the skin and subcutaneous tissue occurs in a variety of rheumatic diseases, being most commonly associated with scleroderma, CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia), dermatomyositis, and overlap syndromes but is a rare complication of systemic lupus erythematosus (SLE). Calcinosis is classified into four subsets: dystrophic, metastatic, idiopathic, or calciphylaxis/iatrogenic. The pathophysiology of calcinosis cutis remains unclear. Our patient developed extensive areas of calcifications in the trunk and extremities (calcinosis universalis) 8 years after SLE diagnosis, which would correspond to a form of dystrophic calcification. No response was observed after treatment with oral diltiazem for 3 months. We review the literature on the pathogenesis and prevalence of calcinosis universalis in SLE.
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PMID:Calcinosis cutis universalis in a patient with systemic lupus erythematosus. 1590 14

Calcinosis universalis is characterized by the deposit of calcium salts in skin, subcutaneous tissue, tendons and muscles. Most cases become apparent during the first decade of life. Clinical aspects may vary from arthralgia to movement limitation, with calcification of soft tissues. Differential diagnosis should exclude fibrodysplasia ossificans progressive, progressive osseous heterodysplasia, myositis ossificans and dermatopolymyositis. There is no specific treatment, but the use of calcium chelates (EDTA), biphosphonates (disodium etidronate) and steroids are mentioned. This paper presents a review of the literature and adds a new case of calcinosis universalis and its evolution in 28 months, describing laboratory and radiograph findings and suggesting the differential diagnosis among processes of soft tissue calcification.
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PMID:Calcinosis universalis: a rare diagnosis. 1593 Oct 36

Glucocorticoids are used for the treatment of inflammatory and autoimmune diseases, cancer, and in prevention of organ rejects. A frequent secondary effect of longterm treatment with corticoids is the loss of bone mass, caused by several mechanisms: decrease in the intestinal calcium absorption, increase of the renal calcium excretion at the distal renal tubule, suppressive effect on the osteoblast and also in apoptosis of osteoclasts, inhibition in local production of IGF I (Insulin-like growth factor) and IGFBPs (binding IGF I proteins necessary for bone metabolism), and decrease on osteocalcin production. Longterm treatment with corticoids is associated with osteoporosis and vertebral fractures. To improve this condition, treatment with bisphosphonates has been proposed. We present here a clinical case of a girl with dermatomyositis and severe osteoporosis with vertebral crushes, who responded well to oral bisphophonate treatment.
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PMID:[Severe osteoporosis with vertebral crushes in juvenile dermatomyositis. Effect of oral alendronate therapy]. 1740 22

We describe the clinical features, therapies, and clinical course of pulmonary arterial hypertension (PAH) in a group of Filipinos with connective tissue diseases (CTDs). We retrospectively reviewed the records of patients diagnosed with PAH by a two-dimensional echocardiogram as a tricuspid regurgitant jet of more than 25 mmHg. All patients had underlying CTDs, defined by the American College of Rheumatology criteria, and were seen at the rheumatology clinics of the University of Santo Tomas Hospital and the St. Luke's Medical Center, Philippines. Of the 33 patients (32 women) included in the analysis, there were 14 patients with systemic lupus erythematosus (SLE), 12 with scleroderma, 5 with mixed connective tissue disease (MCTD), 1 with primary antiphospholipid syndrome (APS), and 1 with dermatomyositis. The average age at PAH diagnosis was 38 +/- 14 years (mean +/- SD), and the mean duration of illness from CTD to PAH diagnosis was 53 +/- 52 months. Twelve patients had died at the time of this report, with a median duration of 15 months (range 1-57 months) from PAH diagnosis to mortality: six of these had scleroderma, five with SLE, and one with APS. The following therapies were used in this group of patients: low molecular weight heparin, warfarin, calcium-channel blockers, aspirin, cyclophosphamide, bosentan, iloprost, and sildenafil. We have described the clinical profile of PAH in a group of Filipino patients with CTDs, most commonly SLE. Various forms of pharmacologic therapies were used among these patients. Mortality remains high, particularly among those with underlying scleroderma. Early recognition and treatment are crucial in order to provide a better outcome for these patients.
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PMID:Pulmonary arterial hypertension among Filipino patients with connective tissue diseases. 1756 78

Ultrasound features of a fluid-calcium level in a soft-tissue mass in the thigh in dermatomyositis are described. As the mass was tender, a soft-tissue abscess required to be excluded by aspiration.
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PMID:Fluid-calcium level in the thigh versus clinical diagnosis of a soft-tissue abscess: ultrasound features of dermatomyositis. 1787 68

The objective of this study was to determine the impact of glucocorticoid exposure on lumbar spine bone mineral density (BMD) in children while concurrently measuring their calcium intake, serum 25-OH vitamin D levels, and physical activity. Forty-three patients (4-18 years) with renal glomerular diseases, dermatomyositis, inflammatory bowel disease, juvenile rheumatoid arthritis, post-solid organ transplant, and Duchenne muscular dystrophy were studied. All received at least 5 mg per day of prednisone for more than 6 months. The mean BMD z score was 0 +/- 0.2 (range, -3.8 to +3.3) with 2 patients (5%) having z scores less than -2. The mean daily calcium intake was 1147 +/- 145 g, with 1 patient having hypovitaminosis D (<15 ng/mL). The mean physical activity level was 7.8 +/- 0.8 h/wk. The small reductions in BMD observed in our population suggest that screening is likely not warranted in all children with chronic glucocorticoid exposure.
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PMID:Bone mineral density in children exposed to chronic glucocorticoid therapy. 1837 41

Calcinosis has long been associated with autoimmune disease and has a distinctive profile in scleroderma, dermatomyositis, systemic lupus erythematosus, and overlap syndromes. However, there have also been a number of case studies of calcific uremic arteriolopathy, or calciphylaxis, described within vessels, including patients with chronic renal insufficiency and several forms of vasculitis. Interestingly, the calciphylaxis associated with vasculitis appears to be unique, although relatively uncommon and is likely secondary to a disruption in the calcium-phosphate-parathyroid hormone axis. However, there appears to be an additional trigger, given that calciphylaxis is seen both in the absence of chronic kidney disease, and in the absence of a deranged calcium-phosphate-parathyroid hormone axis. These additional triggers include a high female predominance, obesity, diabetes and, possibly, warfarin use. In this review, we describe the clinical features of calciphylaxis, particularly in the context of autoimmune disease.
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PMID:Recognizing calcific uremic arteriolopathy in autoimmune disease: an emerging mimicker of vasculitis. 1877 19

We report a case of Epstein Barr virus-associated large B cell lymphoproliferative disorder, with an abdominal cutaneous localization, in an adult treated for 10 years with immunosuppressive agents for a dermatomyositis. This is the third case of immunosuppressive induced lymphoproliferative disorder localized to skin in a patient with dermatomyositis. Diagnosis was unexpectedly obtained by the histologic examination of surgical samples of skin necrosis possibly induced by edetate calcium disodium subcutaneous injections in calcinosis cutis.
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PMID:[Cutaneous EBV-associated lymphoproliferative disorder in a dermatomyositis treated with immunosuppressive agents]. 1902 95

Subcutaneous calcifications occur in a variety of diseases, including juvenile dermatomyositis. These calcifications cause disabling symptoms that do not always respond to immunosuppressant therapy. The calcium antagonist diltiazem reduces subcutaneous calcifications in CREST syndrome and in isolated cases of children with dermatomyositis. Our study was performed to determine the effects of diltiazem when used as adjunctive therapy in children with dermatomyositis.
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PMID:Diltiazem in the treatment of calcinosis in juvenile dermatomyositis. 1907 35

A pronounced linear eruption with ulcerations and calcium extrusion present in a boy with a mild generalized rash clinically consistent with juvenile dermatomyositis or overlap syndrome is reported. Loss of heterozygosity (LOH) is a postzygotic mechanism by which a heterozygous somatic cell may become homozygous or hemizygous at a given gene locus. Such a mechanism can be suspected when a pronounced segmental manifestation of an acquired skin condition with a polygenic background is found to be superimposed on more or less symmetrically distributed nonsegmental lesions of the same disorder. Alternatively, such a segmental manifestation may reflect heterozygosity for a postzygotic mutation involving an additional gene locus. The severe linear lesions in our patient showed a Blaschko-linear arrangement and were superimposed on mild nonsegmental lesions of either amyopathic dermatomyositis or overlap syndrome. Either LOH or a postzygotic mutation at an additional gene locus may explain the pronounced linear involvement.
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PMID:Pronounced linear calcinosis in a boy with mild dermatomyositis. A further possible example of superimposed segmental manifestation of a polygenic disorder. 1943 20


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