PubMed · 6903726
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Iu K Skripkin. 1980. [Poikilodermatomyositis].. https://pubmed.ncbi.nlm.nih.gov/6903726/
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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.
Dermatomyositis is a chronic inflammatory myopathy with severe prognosis. A 57-year-old woman suffering from dermatomyositis is presented who, in the course of the disease, developed acute spontaneous esophageal rupture due to dermatomyositis involvement of the esophagus. She was successfully treated with total esophagectomy and stomach interposition. This is the first report of spontaneous rupture of esophagus in dermatomyositis.