2 weeks after initiation of carbamazepine therapy a 3 year old boy pre
sented with skin rash, fever, lymphadenopathy, hepatosplenomegaly and
nephritis. After exclusion of infectious diseases our differential dia
gnosis focussed on carbamazepine induced hypersensitivity syndrome. We
found a positive lymphocyte transformation assay and patch test to ca
rbamazepine, indicating a specific response, but no anticarbamazepine
antibody, no circulating immune complexes and no decrease in complemen
t components. The hypersensitivity syndrome refers to a severe hyperse
nsitivity response due to a idiosyncratic reaction. The origin is unkn
own. The similarity to other multisystem disorders is discussed. Discu
ssion: Not only aromatic antiepileptic agents (carbamazepine, phenobar
bital, phenytoin) sulfonamides but as well allopurinol and gold salts
are associated with the syndrome. Withdrawal of the drug and treatment
with corticosteroids usually lead to complete recovery.