Opinion statement
Anticonvulsant hypersensitivity syndrome is a rare and potentially life-threatening delayed-type immune reaction with few proven treatments beyond early recognition and immediate discontinuation of the causative drug. Intravenous immunoglobulin has been shown in some uncontrolled studies to hasten recovery and may prove safer than systemic corticosteroid therapy, which carries a significant risk for increased mortality from sepsis. Identification and counseling of at-risk patients through knowledge of potential drug cross-sensitivity and familial susceptibility may help prevent this dangerous idiosyncratic drug reaction.
Similar content being viewed by others
References and Recommended Reading
Shear NH, Spielberg SP: Anticonvulsant hypersensitivity syndrome: in vitro assessment of risk. J Clin Invest 1988, 82:1826–1832.
Bocquet H, Bagot M, Roujeau JC: Drug induced pseudolymphoma and drug hypersensitivity syndrome (drug rash with eosinophilia and systemic symptoms: DRESS). Semin Cutan Med Surg 1996, 15:250–257.
Ben-Menachem E, Gilland E: Efficacy and tolerability of levetiracetam during 1-year follow-up in patients with refractory epilepsy. Seizure 2003, 12:131–135.
Knowles SR, Shapiro LE, Shear NH: Anticonvulsant hypersensitivity syndrome: incidence, prevention, and management. Drug Safety 1999, 6:489–501.
Chadwick D, Shaw MDM, Foy P, et al.: Serum anticonvulsant concentrations and the risk of drug induced skin eruptions. J Neurol Neurosurg Psych 1984, 47:642–644.
Lamotrigine [package insert]. Research Triangle Park, NC: GlaxoSmithKline; 2006.
Chave TA, Mortimer NJ, Sladden, MJ, et al.: Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol 2005, 153:241–253.
Hebert AA, Ralston JP: Cutaneous reactions to anticonvulsant medications. J Clin Psychiatry 2001, 62:22–26.
Tas S, Simonart T: Management of drug rash with eosinophilia and systemic symptoms (DRESS syndrome): an update. Dermatology 2003, 206:353–356.
Mockenhaupt M, Messenheimer J, Tennis P, et al.: Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. Neurology 2005, 64:1134–1138.
Krauss G: Current understanding of delayed anticonvulsant hypersensitivity reactions. Epilepsy Curr 2006, 6:33–37.
Schlienger RG, Shear NH: Antiepileptic drug hypersensitivity syndrome. Epilepsia 1998, 39:83–87.
Lee WC, Leung JL, Fung CW, et al.: Spectrum of anticonvulsant hypersensitivity syndrome: controversy of treatment. J Child Neurol 2004, 19:619–623.
Metry DW, Jung P, Levy ML: Use of intravenous immunoglobulin in children with Stevens-Johnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics 2003, 112:1430–1436.
Prins C, Kerdel FA, Padilla RS, et al.: Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 2003, 139:26–32.
Tan AW, Thong BY, Yip LW, et al.: High-dose intravenous immunoglobulins in the treatment of toxic epidermal necrolysis: an Asian series. J Dermatol 2005, 32:1–6.
Bachot N, Revuz J, Roujeau JC: Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2003, 139:33–36.
Hyson C, Sadler M: Cross sensitivity of skin rashes with antiepileptic drugs. Can J Neurol Sci 1997, 24:245–249.
Hirsch LJ, Weintraub DB, Buchsbaum R, et al.: Predictors of lamotrigine-associated rash. Epilepsia 2006, 47:318–322.
Smith H, Newton R: Adverse reactions to carbamazepine managed by desensitization. Lancet 1985, 1:753.
Tavernor SJ, Wong ICK, Newton R, et al.: Rechallenge with lamotrigine after initial rash. Seizure 1995, 4:67–71.
Besag FMC, Ng GYT, Pool F: Successful re-introduction of lamotrigine after initial rash. Seizure 2000, 9:282–286.
Beran RG: Cross-reactive skin eruption with both carbamazepine and oxcarbazepine. Epilepsia 1993, 34:163–165.
Bavdekar SB, Muranjan MN, Gogtay NJ, et al.: Anticonvulsant hypersensitivity syndrome: lymphocyte toxicity assay for the confirmation of diagnosis and risk assessment. Ann Pharmacother 2004, 38:1648–1650.
Romano A, Pettinato R, Andriolo M, et al.: Hypersensitivity to aromatic anticonvulsants: in vivo and in vitro cross-reactivity studies. Curr Pharm Des 2006, 12:3373–3381.
Seitz CS, Pfeuffer P, Raith P, et al.: Anticonvulsant hypersensitivity syndrome: cross-reactivity with tricyclic antidepressant agents. Ann Allergy Asthma Immunol 2006, 97:698–702.
Johnson KK, Green DL, Rife JP, et al.: Sulfonamide cross-reactivity: fact or fiction? Ann Pharmacother 2005, 39:290–301.
Khalili B, Bahna SL: Pathogenesis and recent therapeutic trends in Stevens-Johnson syndrome and toxic epidermal necrolysis. Ann Allergy Asthma Immunol 2006, 97:272–280.
Hunger RE, Hunziker T, Buettiker U, et al.: Rapid resolution of toxic epidermal necrolysis with anti-TNF-alpha treatment. J Allergy Clin Immunol 2005, 116:923–924.
Gottlieb AB, Chamian F, Masud S, et al.: TNF inhibition rapidly down-regulates multiple proinflammatory pathways in psoriasis plaques. J Immunol 2005, 175:2721–2729.
Qin YM, Auh S, Blokh L, et al.: TNF-alpha induces transient resistance to fas-induced apoptosis in eosinophilic acute myeloid leukemia cells. Cell Mol Immunol 2007, 4:43–52.
Gennis MA, Vemuri R, Burns EA, et al.: Familial occurrence of hypersensitivity to phenytoin. Am J Med 1991, 91:631–634.
Chung WH, Hung SI, Hong HS, et al.: Medical genetics: a marker for Stevens-Johnson syndrome. Nature 2004, 428:486.
Alfirevic A, Jorgensen AL, Williamson PR, et al.: HLA-B locus in Caucasian patients with carbamazepine hypersensitivity. Pharmacogenomics 2006, 7:813–818.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ting, T.Y. Anticonvulsant hypersensitivity syndrome: Identification and management. Curr Treat Options Neurol 9, 243–248 (2007). https://doi.org/10.1007/s11940-007-0010-9
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11940-007-0010-9