Sweet's syndrome (SS) is characterized by the clinical features of fev
er, leucocytosis, neutrophilia and the sudden onset of asymmetric, oft
en very painful skin lesions and dense dermal infiltrates of mature ne
utrophils without signs of vasculitis. Apart from idiopathic cases the
disease is frequently associated with hematological malignancies, but
it may also be observed in patients with solid tumors, mainly tumors
of the genito-urinary tract. In the past, numerous theories have been
proposed to explain the pathogenesis of this rare disease. SS has been
interpreted as a direct response to mechanical and chemical irritants
, an infectious disease or a disorder of neutrophilic chemotaxis and/o
r phagocytosis, but most often it has been described as a hypersensiti
vity reaction. Each of these theories can account for particular sympt
oms, but none of them reconciles the dominating clinical and laborator
y features of the disease. Furthermore recently published casuistic ob
servations suggest the involvement of certain cytokines in particular
G-CSF and Il-6 in the pathogenesis of the disease, which might explain
many of the observed clinical and laboratory findings. The following
article summarizes these data and gives a review of the current litera
ture.