Increase of a wide range of bioactive substances in an active phase of neuro-Sweet disease

Abstract

We present the case of a patient whose skin findings and human leucocyte antigen (HLA) typing were key findings for the diagnosis of his neuro-Sweet disease. A 55-year-old Japanese man with skin rashes and high fever suddenly developed consciousness disturbance, and brain MRI showed encephalitis and leptomeningitis. Neuro-Behçet disease or microbial infection was initially suspected, but he was eventually diagnosed with neuro-Sweet disease based on his skin rashes and pathology and the presence of HLA-B54 and Cw1. He responded to glucocorticoid and recovered without neurological sequelae. The involvement of cytokines has been implicated in the pathogenesis of Sweet disease, but the number of cytokines assayed in each case report is limited. In our patient’s case, the result of a 27-cytokine assay showed increases in a wide range of bioactive substances including inflammatory cytokines, growth factors and chemoattractants in the active phase, indicating the involvement of multiple cytokines in the pathogenesis of Sweet disease.