Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens-Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN.

Abstract

Pediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Diagnosis of DEN relies on clinical features and a detailed medication history. The primary differential diagnosis is reactive infectious mucocutaneous eruption, which can be clinically distinguished by its disproportionate mucous membrane involvement relative to (sparse or absent) skin lesions. Identification and discontinuation of culprit medications is the mainstay of treatment of DEN. Early initiation of immunomodulatory therapy may prevent progression, reducing maximal disease severity and the risk of sequelae. A checklist approach to detailed management of DEN is proposed.