Case Report
Anticonvulsant hypersensitivity syndrome with severe repercussions in the skin and kidneys
Síndrome de hipersensibilidad por antiepilépticos con repercusión cutanea y renal grave por carbamacepina
Rev Neurol 2004
, 38(12),
1136–1138;
https://doi.org/10.33588/rn.3812.2003621
Abstract
INTRODUCTION Anticonvulsant hypersensitivity syndrome (AHS) is characterised by fever, skin rashes and involvement of the internal organs. Owing to the low frequency with which it appears and its high clinical heterogeneity, it is not always suspected. Moreover, the symptoms often overlap with those of a vasculitis or of an infection. The most commonly associated antiepileptic drugs (AED) are the aromatic agents. We report the case of a female patient who developed AHS with several different AED and presented an especially severe kidney and skin disorder due to carbamazepine (CBZ).
CASE REPORT We describe the case of a 26-year-old woman who, after being diagnosed as suffering from secondarily generalised partial seizures, began treatment with 200 mg/12 hours CBZ. A few weeks later, she developed itchy skins lesions compatible with exanthematic pustulosis, together with acute kidney failure requiring haemodialysis. A biopsy study of the kidney revealed immunoallergic tubulointerstitial nephropathy, which is a lesion that has only very occasionally been reported in relation to CBZ therapy. The patient also presented a moderate rise in the level of transaminases and leukocytosis with eosinophilia. She was discharged from hospital without AED but suffered new seizures and was treated with phenytoin and, later, with valproic acid, both as monotherapy. With these drugs she developed AHS consisting in fever, rashes, eosinophilia and subclinical hepatitis. In epicutaneous tests with anticonvulsants, the three AED presented a positive reading, as well as others. The patient was treated with tiagabine, and there were no further hypersensitivity phenomena and a good control of seizures was achieved.
CONCLUSIONS AHS is an infrequent, but potentially serious, clinical entity and must therefore be suspected in patients taking AED who develop fever, rashes or disorders affecting the internal organs.
CASE REPORT We describe the case of a 26-year-old woman who, after being diagnosed as suffering from secondarily generalised partial seizures, began treatment with 200 mg/12 hours CBZ. A few weeks later, she developed itchy skins lesions compatible with exanthematic pustulosis, together with acute kidney failure requiring haemodialysis. A biopsy study of the kidney revealed immunoallergic tubulointerstitial nephropathy, which is a lesion that has only very occasionally been reported in relation to CBZ therapy. The patient also presented a moderate rise in the level of transaminases and leukocytosis with eosinophilia. She was discharged from hospital without AED but suffered new seizures and was treated with phenytoin and, later, with valproic acid, both as monotherapy. With these drugs she developed AHS consisting in fever, rashes, eosinophilia and subclinical hepatitis. In epicutaneous tests with anticonvulsants, the three AED presented a positive reading, as well as others. The patient was treated with tiagabine, and there were no further hypersensitivity phenomena and a good control of seizures was achieved.
CONCLUSIONS AHS is an infrequent, but potentially serious, clinical entity and must therefore be suspected in patients taking AED who develop fever, rashes or disorders affecting the internal organs.
Resumen
Introducción El síndrome de hipersensibilidad por antiepilépticos (SHA) se caracteriza por fiebre, rash cutáneo y afectación visceral. Su poca frecuencia, junto con su gran heterogeneidad clínica, hace que no siempre se sospeche. Además, los síntomas frecuentemente se superponen a una vasculitis o a un cuadro infeccioso. Los fármacos antiepilépticos (FAE) asociados con mayor frecuencia son los aromáticos. Presentamos una paciente que desarrolló un SHA con varios FAE, y fue especialmente grave la afectación renal y cutánea por carbamacepina (CBZ) grave.
Caso clínico Mujer de 26 años que, tras el diagnóstico de crisis parcial secundariamente generalizada, comienza tratamiento con CBZ 200 mg/12 horas. Pocas semanas más tarde desarrolla lesiones cutáneas pruriginosas compatibles con pustulosis exantemática, junto con fallo renal agudo y precisa hemodiálisis. La biopsia renal muestra nefropatía tubulointersticial inmunoalérgica. Esta lesión se ha descrito en escasas ocasiones en relación con el tratamiento con CBZ. Presentaba discreta elevación de transaminasas y leucocitosis con eosinofilia. Se le da de alta sin FAE, pero sufre nuevas crisis, por lo que se trata con fenitoína y, posteriormente, ácido valproico, ambos en monoterapia; desarrolla con dichos fármacos un SHA consistente en fiebre, rash, eosinofilia y hepatitis subclínica. En las pruebas epicutáneas con antiepilépticos, presentan una lectura positiva los tres FAE, además de otros. Se trató con tiagabina, no hay nuevos fenómenos de hipersensibilidad y buen control de las crisis.
Conclusión El SHA es una entidad infrecuente, pero potencialmente grave, por lo que es imprescindible sospecharlo en pacientes con FAE que desarrollen fiebre, rash, y/o afectación visceral.
Caso clínico Mujer de 26 años que, tras el diagnóstico de crisis parcial secundariamente generalizada, comienza tratamiento con CBZ 200 mg/12 horas. Pocas semanas más tarde desarrolla lesiones cutáneas pruriginosas compatibles con pustulosis exantemática, junto con fallo renal agudo y precisa hemodiálisis. La biopsia renal muestra nefropatía tubulointersticial inmunoalérgica. Esta lesión se ha descrito en escasas ocasiones en relación con el tratamiento con CBZ. Presentaba discreta elevación de transaminasas y leucocitosis con eosinofilia. Se le da de alta sin FAE, pero sufre nuevas crisis, por lo que se trata con fenitoína y, posteriormente, ácido valproico, ambos en monoterapia; desarrolla con dichos fármacos un SHA consistente en fiebre, rash, eosinofilia y hepatitis subclínica. En las pruebas epicutáneas con antiepilépticos, presentan una lectura positiva los tres FAE, además de otros. Se trató con tiagabina, no hay nuevos fenómenos de hipersensibilidad y buen control de las crisis.
Conclusión El SHA es una entidad infrecuente, pero potencialmente grave, por lo que es imprescindible sospecharlo en pacientes con FAE que desarrollen fiebre, rash, y/o afectación visceral.
Keywords
Anticonvulsant hypersensitivity syndrome
Antiepileptic drugs
Carbamazepine
Tubulointerstitial nephritis
Palabras Claves
Carbamacepina
Fármacos antiepilépticos
Nefritis tubulointersticial
Pustulosis
Síndrome de hipersensibilidad por antiepilépticos