Clinical Pediatrics: Open Access
Open Access

Abstract

DRESS following FIRES: A Clinical Conundrum

Amanda R. Dahl, Erin E. Knoebel, and Robin M. Lloyd

A previously healthy 2-year old Hmong girl presented to the Emergency Department with first time seizures during a febrile illness. Seizures continued for the next two weeks despite multiple antiepileptic agents. All initial cultures and evaluations were negative, and febrile infection related epilepsy syndrome (FIRES) was diagnosed. Three weeks into her hospital course, the patient developed a morbilliform rash on her thigh that became generalized. Concurrently, she developed fever and tachycardia. Laboratory studies demonstrated eosinophilia, an increase in ALT, AST, and direct/total bilirubin. CMV, EBV, and HHV-6 were negative. Skin biopsy showed lichenoid interface dermatitis. Clinical picture was suggestive of drug reaction with eosinophilia and systemic symptoms (DRESS). Topical and systemic steroids were started. Potential triggers for DRESS were discontinued. The patient deteriorated and cardiorespiratory failure occurred. She required ECMO for eight days. Patient gradually improved over the following weeks despite recurrent seizures and remained in the hospital for 283 days. FIRES is a rare and highly morbid condition in which patients develop status epilepticus in the setting of febrile illness that is often intractable to anticonvulsants and steroids. Typically no causative infection is identified. DRESS, another rare and potentially fatal condition, is a drug hypersensitivity reaction that includes eosinophilia, characteristic skin findings and potential involvement of liver, lungs, kidneys, or other organs. Allopurinol, antiepileptic agents, and antibiotics are the most frequently reported triggers. In this case report, we describe a patient who developed DRESS following FIRES, a sequence not previously described in the literature.