Neuro-Sweet disease in a Japanese woman with Sjögren's syndrome

Sweet disease (SD) is a multisystem inflammatory disorder characterised by fever, cutaneous erythematous plaques and aseptic neutrophilic infiltration of various organs. Neuro-Sweet disease (NSD) is a known rare central nervous system complication of SD. We describe a case of a 39-year-old Japanese woman who was diagnosed as NSD associated with Sjögren's syndrome. She was successfully treated with systemic corticosteroid therapy.