A 60-year-old man with dermatomyositis was admitted to our hospital be
cause of dyspnea and hypertension. He had high fever and convulsive se
izures after admission. Laboratory examinations showed hemolytic anemi
a, thrombocytopenia, and renal failure. A clinical diagnosis of thromb
otic thrombocytopenic purpura (TTP) was made. He failed to respond to
plasma exchange therapy, pulse therapy with methylprednisolone, high-d
ose gamma-globulin therapy, and antiplatelet therapies with ticlopidin
e, dipyridamole and a prostacyclin analog of beraprost sodium. He died
on his 17th day in hospital. Autopsy examination revealed widespread
microthrombi in his kidneys, lungs, spleen, and intestine. Only seven
cases of dermatomyositis or polymyositis complicated by TTP have been
cited in the literature. TTP was fatal in 6 of these 7 cases. Early di
agnosis and prompt treatment may improve the outcome of TTP patients w
ith dermatomyositis. Dermatologists should keep in mind that TTP occas
ionally arises as a serious complication of dermatomyositis.