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Does this DRESS Make Me Look… How to Diagnose and Manage DRESS

August 3, 2022

Written by John Korducki

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This article reviews the literature regarding drug reaction with eosinophilia and systemic symptoms (DRESS) and focuses on the EM and critical care physician’s perspective, summarizing the presentation, clinical features, and management.

Why does this matter?
DRESS is a severe drug reaction with a mortality rate of nearly 10%, with a large and variable constellation of symptoms that require physician awareness, suspicion, and a thorough clinical history to identify. Can you spot it?

Know DRESS and stress less
DRESS is a severe drug related hypersensitivity reaction currently without a definitive pathophysiology. Theories include deficiency in epoxide hydroxylase (detoxifies certain medications), predisposition based on certain HLA haplotypes, reactivation of certain Herpesviridae (commonly HHV-6), T-cell mediated hypersensitivity, or a combination of the above.

DRESS presents as a diffuse exanthematous, macular-papular rash that typically begins on the trunk. 79% of patients will have >50% total body surface area involvement. Fever is another hallmark present in 72-100% of patients. Facial edema and lymphadenopathy (don’t skip your lymph node exam!) are also common features. Mucosal involvement is possible.  Less common features, seen in more severe presentations, are pulmonary involvement (cough, dyspnea, even ARDS) and cardiac involvement.

DRESS can be difficult to identify due to highly variable intervals of time from drug exposure to presentation (1-12 weeks), which makes a thorough clinical and medication history imperative. Many medications can cause DRESS, with vancomycin being the most common – though this is likely due to the frequency with which it’s used.

High risk medications:

  • Allopurinol
  • Anti-tuberculosis medications
  • Anti-epileptics (carbamazepine, lamotrigine, oxcarbazepine, phenytoin, phenobarbital)
  • Mexiletine
  • Minocycline
  • Nevirapine
  • Vancomycin

Low risk medications:

  • Beta-lactams
  • Fluoxetine
  • Imatinib
  • NSAIDs
  • Omeprazole
  • Raltegravir
  • Sorafenib
  • Vemurafenib

Again, diagnosis is largely based on a detailed exam and clinical history, but certain lab tests can also clue you in to DRESS. Eosinophilia is a disease hallmark, but is not present in every patient. Leukocytosis and thrombocytosis are also common. Importantly, LFT derangements are present in 51-100% of cases and can help differentiate it from other rashes and drug eruptions. Clinical decision tools exist, with RegiSCAR being the most complex and sensitive. Practically speaking, if you are concerned about DRESS, the most important step is involving a dermatologist for more definitive testing.

Management is with supportive care, removal of any potentially causative medication, and use of steroids. Avoidance of empiric antibiotics is also recommended due to potential cross-reactivity. Prednisone initiated at a minimum of 1mg/kg/day with a 3-6 month taper is first line therapy, with IV steroids, IVIG, plasmapheresis, and immunosuppressants reserved for refractory cases.

Source
Drug reaction with eosinophilia and systemic symptoms: An emergency medicine focused review. Am J Emerg Med. 2022 Jun;56:1-6. doi: 10.1016/j.ajem.2022.03.024. Epub 2022 Mar 18.

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