We report a case of the uncommon association of dermatomyositis and pr
imary 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 co
nfirmation on muscle biopsy, Diagnosis of primary biliary cirrhosis wa
s made because of elevated values of alcaline phosphatase and gamma gl
utamyl 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 b
e involved.