Anticonvulsant Hypersensitivity Syndrome
Anticonvulsant Hypersensitivity Syndrome
We describe the case of a patient in whom anticonvulsant hypersensitivity syndrome developed during treatment with phenytoin and progressed when therapy was changed to phenobarbital. Although therapeutic options remain controversial, corticosteroids and IV immunoglobulin were used in our patient. The patient had a complete recovery, suggesting the potential benefit of corticosteroids and IV immunoglobulin for anticonvulsant hypersensitivity syndrome.
Anticonvulsant hypersensitivity syndrome (AHS) is a rare but potentially life-threatening adverse drug reaction associated with the aromatic anticonvulsant drugs phenytoin, phenobarbital, primidone, and carbamazapine. This serious reaction is characterized by a triad of fever, skin eruption, and internal organ involvement (ie, hepatitis, nephritis, lymphadenopathy). The rash or skin eruption can range from a mild exanthematous eruption to the more serious Stevens-Johnson syndrome and is present in approximately 90% of patients with this syndrome. Most cases occur 1 to 8 weeks after exposure to the drug and are manifested by fever and malaise accompanied by pharyngitis and cervical lymphadenopathy. Early discontinuance of the offending agent is essential to avoid syndrome progression. In addition, future avoidance of all aromatic anticonvulsants should be considered because cross-reactivity may be as high as 75%. Although medical care is mostly supportive, other treatment modalities include the controversial use of glucocorticoids and anecdotal reports of plasmapheresis, cyclophosphamide, cyclosporine, and IV immunoglobulin. We report the case of a patient who had AHS as a result of a cross-sensitivity reaction to phenytoin and phenobarbital. The clinical manifestations improved during steroid and immunoglobulin therapy and then recurred after withdrawal of glucocorticoids. After the reinstitution of glucocorticoids with a prolonged taper, signs and symptoms resolved completely.
We describe the case of a patient in whom anticonvulsant hypersensitivity syndrome developed during treatment with phenytoin and progressed when therapy was changed to phenobarbital. Although therapeutic options remain controversial, corticosteroids and IV immunoglobulin were used in our patient. The patient had a complete recovery, suggesting the potential benefit of corticosteroids and IV immunoglobulin for anticonvulsant hypersensitivity syndrome.
Anticonvulsant hypersensitivity syndrome (AHS) is a rare but potentially life-threatening adverse drug reaction associated with the aromatic anticonvulsant drugs phenytoin, phenobarbital, primidone, and carbamazapine. This serious reaction is characterized by a triad of fever, skin eruption, and internal organ involvement (ie, hepatitis, nephritis, lymphadenopathy). The rash or skin eruption can range from a mild exanthematous eruption to the more serious Stevens-Johnson syndrome and is present in approximately 90% of patients with this syndrome. Most cases occur 1 to 8 weeks after exposure to the drug and are manifested by fever and malaise accompanied by pharyngitis and cervical lymphadenopathy. Early discontinuance of the offending agent is essential to avoid syndrome progression. In addition, future avoidance of all aromatic anticonvulsants should be considered because cross-reactivity may be as high as 75%. Although medical care is mostly supportive, other treatment modalities include the controversial use of glucocorticoids and anecdotal reports of plasmapheresis, cyclophosphamide, cyclosporine, and IV immunoglobulin. We report the case of a patient who had AHS as a result of a cross-sensitivity reaction to phenytoin and phenobarbital. The clinical manifestations improved during steroid and immunoglobulin therapy and then recurred after withdrawal of glucocorticoids. After the reinstitution of glucocorticoids with a prolonged taper, signs and symptoms resolved completely.
