Drug rash with eosinophilia and systemic symptoms syndrome in an adolescent - efficiency of immunoglobulin G in a corticosteroid resistant case

Abstract

Introduction. Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome (sy) to carbamazepine has a heterogeneous clinical presentation. The aim of this report is to indicate the efficacy of immunoglobulin G in the treatment of corticosteroid resistant DRESS sy. Case outline. A adolescent suffering from epilepsy treated with carbamazepine and Na-valproate was hospitalized for fever, elevated transaminases, lymphadenopathy, splenomegaly. Every day there was an eruption of skin efflorescence. On the sixth day of hospitalization, the number of eosinophils increased to 24% (780/ml absolute number). There was no desired response to methylprednisolone during the first eight days of treatment or prednisolone during further treatment, with concomitant administration of antihistamines from day one of hospitalization, Na-valproate, metformin hydrochloride, elimination diets, and carbamazepine withdrawal. Significant clinical, hematologic and biochemical improvement occurred the day after the first dose of intravenous immunoglobulin G (IVIG). Conclusion. We point out the need to change the DRESS sy treatment recommendations in favor of the IVIG (as soon as possible, already third or fourth day of treatment) in patients in whom the treatment with corticosteroids has no effect. Until new cases of the proven role of IVIG in the treatment of DRESS sy are published, corticosteroids remain the first therapeutic choice.