Abstract
Hemophagocytic syndrome（HPS）is a very rare complication of juvenile dermatomyosits（JDM）．
We report a case of JDM with HPS． A l4-yr-old girl was admitted to our hospital complaining of remittent fever，
appetite loss and body weight loss． She could not walk with arthralgia of the knee． Initially， she was suspected
of juvenile idiopathic arthritis． We administered ibuprofen． Her appetite loss and the pain of knee immediately
disappeared． However she showed heliotorope rash and Gottron sign. We finally make a diagnosis of JDM．
Although we treated with ibuprofen for two weeks， her signs and symptoms relapsed． Laboratory examinations
revealed anemia，decreased number of platelets and elevated transaminases．Bone marrow aspiration showed
hemophagocytosis，indicating complicated HPS． Methylprednisolone pulse therapy was effectively administered．
It is seemed that steroid pulse therapy is effective therapy against HPS with JDM．
