Treatment of DRESS Syndrome

Initial Management

The American College of Oncology recommends immediate discontinuation of the suspected causative drug as the first and most crucial step in managing DRESS syndrome, with prompt dermatology consultation for all suspected cases 1, 2, 3, 4
A complete workup is necessary, including total body skin examination, complete blood count with differential, liver and kidney function tests, urinalysis, blood cultures, and skin biopsy if diagnosis is uncertain, as recommended by the American College of Oncology 1

For severe DRESS syndrome, the American College of Oncology recommends admission to a burn unit or ICU with dermatology and wound care services consultation, initiation of IV methylprednisolone, and provision of supportive care, including fluid and electrolyte balance management, minimizing insensible water losses, infection prevention, topical treatment, and oral antihistamines 1, 5
The American College of Oncology suggests considering intravenous immunoglobulin (IVIG) at a total dosage of 1-2 g/kg in patients not responding to systemic steroids, and cyclosporine may also be considered in severe or steroid-unresponsive cases 5

The American College of Oncology recommends consulting appropriate specialists based on organ involvement, such as ophthalmology, otolaryngology, urology, or gynecology for mucous membrane involvement, and pain or palliative care consultation for symptom management 1, 5
Close monitoring is necessary using serial clinical photography to track skin manifestations, as recommended by the American College of Oncology 1

The American College of Oncology recommends weaning systemic corticosteroids over at least 4 weeks, and prolonged immunosuppression may be necessary in DRESS syndrome due to its T-cell immune-directed toxicity 1

Unlike in Stevens-Johnson syndrome, corticosteroids are not contraindicated in DRESS syndrome, and adequate immunosuppression is necessary with corticosteroids or other agents, as recommended by the American College of Oncology 1
The Centers for Disease Control and Prevention (CDC) recommends avoiding prophylactic use of corticosteroids when initiating medications known to cause DRESS, as this has not proven effective and may increase the risk of skin rash 6
DRESS typically occurs after 6 weeks of treatment with the offending drug, which distinguishes it from other drug reactions, as noted in Liver International 2, 3

Constitutional symptoms, including rigors, myalgias, and arthralgias, are commonly present in patients with DRESS syndrome, according to the British Journal of Pharmacology 7
Nephritis with abnormal kidney function tests may occur in patients with DRESS syndrome, as reported by the British Journal of Pharmacology 7

Urinalysis to evaluate for nephritis is a recommended diagnostic test for patients with suspected DRESS syndrome, as suggested by the Journal of Clinical Oncology 8

Patients with severe symptoms or organ involvement should be admitted to hospital, and consideration should be given to ICU or burn unit admission for severe cases, as recommended by the Journal of Clinical Oncology 8

The presence of lymphocytic CD4+ infiltrates with eosinophils in skin biopsy is a diagnostic feature of DRESS syndrome, as noted by the Annals of Oncology 9
DRESS syndrome can be distinguished from Stevens-Johnson syndrome or toxic epidermal necrolysis by the presence of eosinophilia, longer latency, and organ involvement, as reported by the Annals of Oncology 9