Ethosuximide‐induced Stevens–Johnson syndrome: Beneficial effect of early intervention with high‐dose corticosteroid therapy

Abstract

We report two rare cases of childhood epilepsy patients who developed ethosuximide-induced Stevens-Johnson syndrome (SJS). Unlike typical SJS, the initial eruption of both patients presented well-demarcated, infiltrating firm papules mainly on the cheeks and the extensor aspects of the arms (case 1), and multiple vesicles on the soles and oral aphthosis (case 2), which closely mimicked viral exanthema. We diagnosed both patients with ethosuximide-induced SJS, based on the dosing period and the positive results of drug-induced lymphocyte stimulation test. Systemic corticosteroids are usually selected as a standard therapy for SJS, despite controversial results regarding their effectiveness. In case 1, an i.v. pulse therapy of methylprednisolone (30 mg/kg, 3 days consecutively) was initiated on day 7 from the onset of illness, and an i.v. immunoglobulin (400 mg/kg, 5 days consecutively) was added the following day. In case 2, an i.v. prednisone treatment (1 mg/kg, for 1 week) was initiated on day 4 from the onset. Eventually, the early therapeutic interventions resulted in good outcomes in both patients.

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap triggered by ethosuximide: a rare pediatric report

“…A large European multicenter study 10 and a meta-analysis 11 suggest that a short course of moderate to high systemic corticosteroid therapy (e.g., prednisone 1-2 mg/kg daily for 3-5 days) may have a beneficial effect if administered within the first 24-48 hours of symptoms. Two pediatric cases described in the literature of SSJ induced by ethosuximide on children at the same age 7 suggested that the therapeutic regimen of corticosteroids and/or IVIG may be effective in treating it, especially in the early stage of illness, as well as the clinical course of our patient.…”

“…The diseases SJS/TEN are severe mucocutaneous adverse reactions, and although these conditions are commonly triggered by drugs (risk limited to the first 8 weeks of treatment), they can also have an infectious cause (such as Mycoplasma pneumonia, herpes simplex virus, and Epstein-Barr virus) or be caused by immunization, especially in children 4,7 .…”

“…Children aged 11-15 years and polymedicated patients 2 have the highest incidence. ASD are known to cause SJS 1,5 , but only a very few ethosuximide-induced SJS cases have been reported [5][6][7] .…”

Ethosuximide‐induced Stevens‐Johnson syndrome/toxic epidermal necrolysis overlap confirmed by patch testing in a child

“…However, for specific drugs, such as carbamazepine, sensitivity can be up to 62.5% and its use is supported when there is a clear benefit of the diagnostic information obtained 5 . In all ethosuximide‐induced SJS/TEN cases reported so far, patch testing was performed in only one patient, also at a 10% concentration, with a negative result but a positive drug‐induced lymphocyte transformation test 3 …”

“…Aromatic anticonvulsants are among the most frequent culprits in pediatric SJS/TEN 1,2 . Ethosuximide, a nonaromatic antiepileptic, has been rarely implicated in SJS/TEN, with only six cases of SJS and two cases of TEN reported, exclusively in children, as its main indication are absence seizures 1‐4 …”