2019 Volume 31 Issue 4 Pages 307-313
A 30-year-old man was admitted to our hospital because of spike fever and rash. He had been suffering from facial rash, discoid lupus on his anterior chest wall and polyarthralgia which was exacerbated every summer since 5 years ago. Two years later, he was diagnosed as having acute myocarditis of unknown cause, and thereafter fever sometimes occurred which went down with non-steroidal anti-inflammatory drugs. Because these symptoms became resistant to the previous treatment 3 weeks earlier, he was admitted to our hospital. On admission, there were pancytopenia and elevated level of serum ferritin, and bone marrow findings showed he had hemophagocytic syndrome(HPS)without any evidence of infectious diseases or hematologic malignancy. His symptoms strongly suggested that he had systemic lupus erythematosus(SLE)although his serum anti-nuclear antibody was negative, so lupus-band test of the skin was performed, revealing he had SLE complicated by HPS. This case suggests that histological examination such as lupus band test of the skin should be planned when clinical symptoms suggest occurrence of SLE despite negative serological data.