Abstract

The development of a purpura-fulminans-like disorder which is a human equivalent of a local Shwartzman reaction in a woman with active chronic hepatitis is described. The cyclical appearance of blue-black, well circumscribed, haemorrhagic, acutely painful lesions in the buttocks, over the lateral aspects of the thighs, and on the arms suggested the diagnosis. Evidence of increased intravascular coagulation was obtained although interpretation of clotting factor deficiencies in the presence of parenchymal liver disease was difficult. Treatment with heparin arrested the disorder on three separate occasions. The reasons for the development of the syndrome are not clear and even more surprising was the occurrence of such a disorder in the presence of increased fibrinolysis. While disseminated intravascular coagulation has been described in association with liver disease, the development of features of purpura fulminans in such patients does not appear to have been noted.