PubMed · 5184734
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Poulain. 1968. [Dermatomyositis].. https://pubmed.ncbi.nlm.nih.gov/5184734/
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We report a case of the uncommon association of dermatomyositis and primary biliary cirrhosis in a causasion male of 48-year-old. Diagnosis of dematomyositis was made because of muscle weakness, loss of weight, skin telangiectasia, elevated serum concentration of creatine kinase, polyphasic low amplitudes waves on electromyography and histologic confirmation on muscle biopsy. Diagnosis of primary biliary cirrhosis was made because of elevated values of alcaline phosphatase and gamma glutamyl transferase, elevated values of type II mitochondrial antibody and compatible histological lesions on liver biopsy. We found only ten case reports associating polymyositis and primary biliary cirrhosis. We hypothesize that hepatic and muscle mitochondrial dysfonction may be involved.
Vesicle formation in dermatomyositis is rare. We describe two women with dermatomyositis and vesicle formation on their extremities. Both had an ovarian cancer and histologic examinations revealing subepidermal vesicles. In both patients, direct immunofluorescence did not reveal deposition of immunoglobulin in the basement membrane zone. Of our two patients and the 17 previously reported to have dermatomyositis with vesicle formation, 10 had an internal malignant disease. Of these 10 patients, eight women had gynecologic malignant disease and two men had gastric cancer and lung cancer, respectively. Vesicle formation in dermatomyositis is strongly related to the presence of an internal malignant process, especially gynecologic malignant disease in female patients.
In polymyositis and sporadic inclusion body myositis, clonal expansion of CD8+ cells which are primed to recognize previously unknown muscle antigens occurs. Compared with sporadic inclusion body myositis, however, in which the T-cell response may not be antigen driven, there is in polymyositis an overexpression of certain T-cell receptor gene families among the autoinvasive T-cells. Although studies on the endomysial expression of cytokines and cell adhesion molecules have provided additional support for the concept of an ongoing immune process, the site of sensitization and the mechanism by which the autoimmune process is triggered remains to be established. In dermatomyositis, a multiorgan disease, evidence exists that the complement-mediated microvascular injury by the putative antibody may not be limited to the endomysial vessels but may also involve the blood vessels in the dermis. The antigenic target on the endothelial cell in dermatomyositis patients and the pathogenic role of the recently studied anti-Mi-2 antibody directed against a helicase are still to be determined.