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)

The purpose of this case report is to assess and review the literature to determine the frequency of occurrence of dermatomyositis (DM). Dermatomyositis is a rare autoimmune condition that disproportionately affects adolescence and pediatric patients. The symptomatology experienced in this condition includes but not limited to fatigue, reduced mobility, and dysphagia. Symptoms of dysphonia and dyspnea have been reported due to weakened esophageal and respiratory muscle. Another major complication seen in DM is calcinosis. Calcinosis is a calcium deposit on soft tissue. This is mostly been attributed to late diagnosis or use of ineffective treatment regimen. Systemic corticosteroid is the first-line treatment for DM; however, other agents such as anti-malaria, IVIG, and immunosuppressive therapies have been used successfully.
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PMID:Dermatomyositis: An Acute Flare and Current Treatments. 3124 26

BACKGROUND Increased serum levels of basic calcium phosphate (BCP) and calcium pyrophosphate (CPP) are found in patients on dialysis, following trauma, and are associated with connective tissue diseases (CTDs), including dermatomyositis, scleroderma, and systemic lupus erythematosus (SLE). The shoulder is the joint most commonly associated with BCP crystal periarthritis. A report is presented of a case of BCP crystal periarthritis involving the distal interphalangeal (DIP) joints in a patient with SLE. CASE REPORT A 34-year-old woman with SLE presented with destructive arthritis of the DIP joints that developed during a two-year period, despite immunosuppressive therapy. Aspiration of synovial fluid from a DIP joint showed a lack of inflammatory cells, but the fluid was positive for the presence of crystals on alizarin red S histochemical staining. CONCLUSIONS A case of BCP crystal periarthritis is reported in a patient with SLE with chronic joint symptoms that were unresponsive to immunosuppressive therapy. This case has shown that chronic joint symptoms that are unresponsive to immunosuppressive therapy may be due to causes other than connective tissue disease (CTD) and that imaging studies and diagnostic workup that includes synovial fluid examination may support the diagnosis of BCP crystal periarthritis.
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PMID:Basic Calcium Phosphate Crystal Periarthritis Involving the Distal Interphalangeal Joints in a Patient with Systemic Lupus Erythematosus. 3136 82

Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by muscle weakness and cutaneous manifestations in adults and children. Calcinosis, a complication of DM, is the abnormal deposition of insoluble calcium salts in tissues, including skin, subcutaneous tissue, tendons, fascia, and muscle. Calcinosis is more commonly seen in juvenile DM (JDM), but also develops in adult DM. Although the mechanism of calcinosis remains unclear, several pathogenic hypotheses have been proposed, including intracellular accumulation of calcium secondary to an alteration of the cellular membrane by trauma and inflammation, local vascular ischemia, dysregulation of mechanisms controlling the deposition and solubility of calcium and phosphate, and mitochondrial damage of muscle cells. Identifying calcinosis biomarkers is important for early disease detection and risk assessment, and may lead to novel therapeutic targets for the prevention and treatment of DM-associated calcinosis. In this review, we summarize myositis autoantibodies associated with calcinosis in DM, histopathology and chemical composition of calcinosis, genetic and inflammatory markers that have been studied in adult DM and JDM-associated calcinosis, as well as potential novel biomarkers.
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PMID:Calcinosis Biomarkers in Adult and Juvenile Dermatomyositis. 3223 4

Calcinosis cutis is a deposition of calcium salts in the skin and subcutaneous tissue which can occur in connective tissue diseases such as scleroderma, dermatomyositis, myositis and overlap syndrome, but rarely in association with systemic lupus erythematosus (SLE). It is subdivided into a localized 'circumscripta' and diffuse 'universalis'. The few reported cases of calcinosis in SLE were mainly of the circumscripta type. Calcinosis universalis is extremely rare and is usually associated with a history of chronic active SLE in female patients, with few proven effective treatments. We report a case of a young female patient with a long and complicated history of SLE. She presented with widespread pain and tenderness associated with multiple subcutaneous skin lesions. She was found to have evidence of calcinosis universalis on X-rays. Investigations did not reveal any associated conditions that could explain this diagnosis other than her prolonged history of SLE.
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PMID:Calcinosis cutis universalis in a patient with systemic lupus erythematosus: a case report. 3269 83

Calcinosis cutis is a disorder of pathologic calcium deposition in the cutaneous and subcutaneous layers of skin. While common in dermatomyositis and scleroderma, calcinosis cutis less frequently occurs in systemic lupus erythematosus (SLE) and is infrequently described in literature. In this report, we discuss the case of a 36-year-old patient with SLE, presenting with vascular compromise, ulceration, and superimposed infection of her left hand as a consequence of severe calcinosis cutis. This report includes a review of the current literature, and highlights the importance of early detection and intervention in preventing disease complications.
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PMID:Dystrophic Calcinosis Cutis in Systemic Lupus Erythematosus. 3271 67


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