Successful Treatment of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) with IVIG in pediatric patients ; case series

Keren Smuel Zilberberg 1 Nufar Marcus 1,2 Daniel Landau 1 Moran Almog 1 Dario Preis 4 Rachel Strausberg 3 Oded Scheuerman 1,4
1Department of Pediatrics B, Schneider Children’s Medical Center
2Kipper institute of Immunology, Schneider Children’s Medical Center
3Pediatric neurology unit, Schneider Children’s Medical Center
4Pediatric pulmonology unit, Schneider Children’s Medical Center

Aim: To describe a case series of pediatric patients with Drug reaction eosinophilia systemic symptoms (DRESS) who were treated successfully with intravenous immune-globulins (IVIG).

Background: DRESS is a rare, potentially life-threatening drug-induced hypersensitivity reaction. The most frequently reported drugs causing DRESS are aromatic antiepileptic agents. Prompt withdrawal of the offending drug is the mainstay of treatment. Systemic corticosteroids is currently the most widely accepted and used treatment. A few case reports of IVIG treatment have been described in the literature with mixed results, mostly in adult patients.

Methods: a retrospective review of children hospitalized in Schneider children medical center with Dress syndrome between 1999-2015 and received IVIG in addition to withdrawal of the offending drug and systemic corticosteroids. The data collected included: demographics, clinical presentation, laboratory findings, treatment protocols and outcome.

Results: 6 patients received IVIG for treatment of DRESS. Mean age was 10.7±5.2 yr. The offending drugs were Carbamazepine, Phenytoin, Lamotrigine and Valporic Acid. Clinical syndrome included fever, rash, lymphadenopathy, dyspnea and hepatic involvement. IVIG dosage was 2 g/kg. After IVIG treatment, fever resolved in 1.5±1.7d, rash disappeared after 6.8 ± 0.75 d. Laboratory results (i.e liver enzyme) improved after 3.8 ±1.6 d. Patients were discharged after 6.8± 2.13 d. There was no mortality .

Discussion: Our study suggest that IVIG administered in DRESS syndrome can hasten recovery, when given in addition to drug withdrawal and systemic corticosteroids . IVIG can be useful even in cases resistant to systemic corticosteroids.

Keren Smuel Zilberberg
Keren Smuel Zilberberg








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