Epstein-Barr infection causing toxic epidermal necrolysis, hemophagocytic lymphohistiocytosis and cerebritis in a pediatric patient
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Abstract
Toxic epidermal necrolysis -the most serious variant of Steven Johnson Syndrome -arises as the result of cell-mediated cytotoxic reaction against keratinocytes. Most common inciting factors include drugs, and infections. On the other hand, Hemophagocytic lymphohistiocytosis (HLH), is a syndrome characterized by enormous immune response in the absence of down-regulation of activated immune cells resulting in cytokine storm causing severe tissue damage.
Up to date, several cases of concomitance of Toxic Epidermal Necrolysis (TEN) and Hemophagocytic Lympohystiocytosis (HLH) in pediatric patients have been reported. Both situations can be fatal and pediatricians should be aware that these two clinical entities are not mutually exclusive, to the contrary they may coexist.
We herein describe a case of Toxic Epidermal Necrolysis, complicated with Hemophagocytic Lymphohistiocytosis with Central Nervous System involvement due to EBV infection.
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