A 71-year-old woman was presented with 5-year history of recurrent, multiple, painful or tender, erythematous plaques on her lower limbs accompanied by fever and arthralgia. Histopathological findings showed a dense infiltration of mature neutrophils in the upper and mid dermis without leukocytoclastic vasculitis. These clinical and histopathological findings were consistent with Sweet's syndrome. The laboratory investigations demonstrated elevated ESR and IgA gammopathy, but no leukocytosis or neutrophilia. Her eruption revealed good responses to systemic steroid therapy.

We herein describe a patient with Sweet's syndrome without leukocytosis, probably associated with a benign IgA gammopathy.