DRESS Syndrome Management and Treatment

Introduction to DRESS Syndrome

The American College of Physicians recommends immediate discontinuation of the suspected causative drug as the first and most crucial step in managing DRESS syndrome, followed by prompt dermatology consultation and initiation of systemic corticosteroids for all suspected cases 1
DRESS syndrome involves a complex interplay of immunologic and genetic factors through the major histocompatibility complex (MHC), with certain genetic predispositions strongly associated with specific drug reactions 2

The pathophysiology of DRESS syndrome involves reactivation of herpes family viruses and activation of lymphocytes, leading to a delayed hypersensitivity reaction, with T-cell immune-directed toxicity playing a central role 1
The reaction typically occurs after a latent period of 2-6 weeks following exposure to the offending drug, distinguishing it from other drug reactions 1
Cutaneous manifestations of DRESS syndrome include a morbilliform (maculopapular) confluent rash, which is the most common presentation, and the rash typically involves more than 30% of body surface area 2, 3
Systemic manifestations include fever, constitutional symptoms, lymphadenopathy, hematologic abnormalities, and multi-organ involvement, including hepatitis, pneumonitis, myocarditis, pericarditis, and nephritis 2

The diagnosis of DRESS syndrome is clinical, based on exposure to the causative drug, cutaneous eruption, systemic involvement, hematologic abnormalities, and internal organ involvement 1, 2
DRESS syndrome must be distinguished from other severe cutaneous adverse reactions, including Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), Acute Generalized Exanthematous Pustulosis (AGEP), and other drug hypersensitivity syndromes 2, 4, 5

The European Society of Clinical Microbiology and Infectious Diseases recommends first-line management of DRESS syndrome, including immediate discontinuation of the suspected causative drug, prompt dermatology consultation, and initiation of systemic corticosteroids 1
Systemic therapy includes initiation of systemic corticosteroids, such as IV methylprednisolone 1-2 mg/kg/day, and weaning over at least 4 weeks 1
Supportive care includes fluid and electrolyte balance management, minimizing insensible water losses, infection prevention measures, and topical treatment and oral antihistamines for symptomatic relief 1

The Journal of Allergy and Clinical Immunology notes that relapse can occur in approximately 12% of cases, particularly with DRESS associated with viral reactivation 5
Genetic testing, such as HLA-B*5701 for abacavir, may be useful before prescribing certain medications to prevent hypersensitivity reactions 2
Patch testing or intradermal testing should be avoided until at least 6 months after the acute reaction and at least 1 month after discontinuation of systemic corticosteroids 5