Systemic lupus erythematosis (SLE) is an autoimmune disorder that may
be associated with antiepileptic drugs (AEDs). The postulated mechanis
ms of drug-related lupus (DRL) include either an unmasking of idiopath
ic SLE or the actual induction of autoantibodies. Although ethosuximid
e (ETH) is one of the drugs most implicated in childhood DRL, our trea
tment of a 13-year-old girl with ETH-associated DRL prompted a literat
ure search that revealed only 15 other cases. Many of these were on mu
ltiple AEDs. We subsequently became aware of two other cases. The rela
tionship of ETH to SLE in these three children is discussed, and clini
cal and laboratory features useful in the differentiation of various t
ypes of SLE are reviewed.