Treatment of anticonvulsant hypersensitivity syndrome with intravenous immunoglobulins and corticosteroids

Anticonvulsant hypersensitivity syndrome is a specific severe idiosyncratic reaction to the aromatic antiepileptic drugs. The most frequent presenting symptoms are fever and rash, lymphadenopathy owing to lymphoid hyperplasia, hepatitis, and interstitial nephritis. Severe skin reactions (Stevens-Johnson syndrome or toxic epidermal necrolysis) have also been reported. Early detection is crucial owing to the high mortality rate. Although withdrawal of the offending drug is critical, the optimal treatment approach remains controversial. Previous studies report a severe course and prolonged hospital stay for cutaneous drug-related reactions, including referral to a burn center, skin débridement, and allograft skin coverage. The aim of the present report is to describe four adolescents with antiepileptic drug hypersensitivity syndrome who were treated with intravenous immunoglobulin and systemic corticosteroids. All recovered completely following an uncomplicated and relatively short course and hospitalization. Findings indicate that this regimen might be a promising treatment option in this patient population. Larger, controlled trials are needed to reach a definitive conclusion.