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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

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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.

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Authors and Affiliations

  1. Division of Community Pediatrics, Department of Pediatrics, Alberta Children’s Hospital, 28 Oki Dr NW, Calgary, AB, T3B6A8, Canada

    Michele L. Ramien

  2. Division of Dermatology, Department of Medicine, University of Calgary, Calgary, AB, Canada

    Michele L. Ramien

  3. Department of Dermatology, University of British Columbia, Vancouver, BC, Canada

    Danny Mansour

  4. Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, ON, Canada

    Neil H. Shear

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Correspondence to Michele L. Ramien.

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Michele Ramien has received research funding from the Pediatric Dermatology Research Alliance for a project on pediatric SJS-TEN. Danny Mansour and Neil Shear have no conflicts of interest to declare.

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Ramien, M.L., Mansour, D. & Shear, N.H. 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. Pediatr Drugs 24, 307–319 (2022). https://doi.org/10.1007/s40272-022-00515-0

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