Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome manifests as a significant, medication-induced, adverse reaction with cutaneous, hematological and multi-organ involvement. Sero-negative autoimmune encephalitis is inflammation of the brain secondary to autoimmune processes in the absence of an identifiable autoantibody. Case presentation: We present a pediatric case of sero-negative autoimmune encephalitis who subsequently developed DRESS syndrome as a result of exposure to ceftriaxone, omeprazole or acyclovir. She did not respond to either high-dose steroids or intravenous immunoglobulins (IVIG). Plasmapheresis was successful in treating symptoms relevant to both DRESS and sero-negative autoimmune encephalitis. No side effects were encountered. Conclusion: To our knowledge, plasmapheresis has only been reported once to treat DRESS syndrome in a pediatric patient. Early recognition and treatment of this syndrome is paramount. We suggest that plasmapheresis is a relatively safe alternative treatment option for resistant cases especially within the context of other autoimmune conditions.

Keywords: Plasmapharesis, DRESS syndrome, ceftriaxone, omeprazole, aciclovir, sero--negative autoimmune encephalitis, case report