Rendu-Osler-Weber-like telangiectasia associated with primary biliary cirrhosis

An otherwise asymptomatic woman presented with the recent onset of telangiectasias of the lips and fingertips, and without a family history of similar lesions. Results of laboratory tests included elevated liver function, positive antimitochondrial antibodies, and microscopic findings compatible with the diagnosis of primary biliary cirrhosis (PBC). New lesions of skin and mucous membranes resembling manifestations of the Rendu-Osler-Weber syndrome should prompt the physician to rule out PBC and associated autoimmune diseases.