The diagnosis of Sweet's syndrome rests on a combination of clinical symptoms and characteristic physical and pathologic features. Patients typically have fever and tender erythematous skin lesions (papules, nodules, or plaques). Neutrophilia, high levels of serum inflammatory markers, and diffuse mature neutrophil infiltration localized to the upper dermis are the most important findings. Sweet's syndrome was first described by Robert Sweet in 1964, whose sentinel paper described 8 women with fever, leukocytosis, and erythematous plaques infiltrated by neutrophils. Subsequently, extracutaneous sites were included in the diagnosis. This review of publications between 1964 and April 2012 found 1683 reports of Sweet's syndrome of which only 8 were published between 1964 and 1969, after which the number of the papers grew by decades to 59, 228,459 and 692. With more articles, there are more reports of malignancy-associated Sweet's syndrome. This may reflect the awareness by physicians of the disease and of the drugs which may cause it. There is considerable overlap in the constitutional findings of Sweet's syndrome and malignant disorders. It is crucial that the possibility of Sweet's syndrome be included in a hematologist or oncologist's differential diagnosis of fever and skin lesions. (C) 2012 Elsevier Ireland Ltd. All rights reserved.