SCHNITZLERS-SYNDROME VERSUS ADULT-ONSET STILLS-DISEASE

We report the case of a 50-year-old man with a 4-year history of high spiking fever accompanied by a widespread, urticarial, non-pruritic or only sometimes mildly pruritic eruption and arthralgia. He also had g eneralized lymphadenopathy, hepatosplenomegaly, and hyperosteoses of t he lower lumbar spine. Laboratory examination revealed an elevated ery throcyte sedimentation rate, elevated white blood cell and platelet co unts, hypoalbuminemia, and elevated serum IgM with IgM kappa monoclona l immunoglobulin. We diagnosed his condition as Schnitzler's syndrome, in contrast to the diagnosis of adult onset Still's disease, for whic h he had been initially followed up by his internist. We compare clini cal and histopathological findings for both diseases and, as this pati ent meets two of the six existing diagnostic criteria for adult onset Still's disease, we propose that Schnitzler's syndrome is an important entity to be added to the list of differential diagnoses for adult on set Still's disease.