Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms. Part II diagnosis and management - 11/04/24

Doi : 10.1016/j.jaad.2023.02.073

Brian M. Wei, BS ^a, Lindy P. Fox, MD ^b, Benjamin H. Kaffenberger, MD, MS ^c, Abraham M. Korman, MD ^c, Robert G. Micheletti, MD ^d, Arash Mostaghimi, MD, MPA, MPH ^e, Megan H. Noe, MD, MPH, MSCE ^e, Misha Rosenbach, MD ^d, Kanade Shinkai, MD, PhD ^b, Jason H. Kwah, MD, MSc ^f, Elizabeth J. Phillips, MD ^g, Jean L. Bolognia, MD ^a, William Damsky, MD, PhD ^h, Caroline A. Nelson, MD ^a,^⁎
^a Department of Dermatology, Yale School of Medicine, New Haven, Connecticut
^b Department of Dermatology, University of California, San Francisco, California
^c Department of Dermatology, The Ohio State University, Columbus, Ohio
^d Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
^e Department of Dermatology, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts
^f Department of Medicine, Section of Rheumatology, Allergy and Immunology, Yale School of Medicine, New Haven, Connecticut
^g Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee
^h Department of Pathology, Yale School of Medicine, New Haven, Connecticut

^∗Correspondence to: Caroline A. Nelson, MD, Department of Dermatology, Yale University School of Medicine, 15 York St, New Haven, CT 06510.Department of DermatologyYale University School of Medicine15 York StNew HavenCT06510

Abstract

Drug-induced hypersensitivity syndrome, also known as drug reaction with eosinophilia and systemic symptoms, is a severe cutaneous adverse reaction characterized by an exanthem, fever, and hematologic and visceral organ involvement. The differential diagnosis includes other cutaneous adverse reactions, infections, inflammatory and autoimmune diseases, and neoplastic disorders. Three sets of diagnostic criteria have been proposed; however, consensus is lacking. The cornerstone of management is immediate discontinuation of the suspected drug culprit. Systemic corticosteroids remain first-line therapy, but the literature on steroid-sparing agents is expanding. Longitudinal evaluation for sequelae is recommended. Adjunctive tests for risk stratification and drug culprit identification remain under investigation. Part II of this continuing medical education activity begins by exploring the differential diagnosis and diagnosis of drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms and concludes with an evidence-based overview of evaluation and treatment.

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Key words : delayed hypersensitivity reaction, DHR, DiHS, DRESS, drug rash, drug-induced hypersensitivity syndrome, drug reaction, drug reaction with eosinophilia and systemic symptoms, eosinophilia, exanthem, exanthematous drug eruption, hypersensitivity, maculopapular eruption, morbilliform eruption, severe cutaneous adverse drug reaction, SCAR

Abbreviations used : ACLE, AGEP, AITL, CMV, CYP, dIDT, DiHS, DRESS, EBV, ELISpot, EM, GVHD, HES, HHV, HLA, HLH, HMGB1, IFN, Ig, IL, IV, IVIG, LTT, PBMC, SCAR, scRNA-seq, SJS/TEN, STAT, TARC

Plan

Introduction

Differential diagnosis

	Funding source: None.
	IRB approval status: Not applicable.
	Patient consent: Consent for the publication of recognizable patient photographs or other identifiable material was obtained by the authors and included at the time of article submission to the journal stating that all patients gave consent with the understanding that this information may be publicly available.
	Date of release: May 2024.
	Expiration date: May 2027.