Drug reaction with eosinophilia and systemic symptoms
Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) is caused by exposure to certain medications that may result in a rash, fever, inflammation of internal organs, lymphadenopathy, and characteristic hematologic abnormalities such as eosinophilia, thrombocytopenia, and atypical lymphocytosis. The syndrome has about a 10% mortality. Treatment consists of stopping the offending medication and providing supportive care. Systemic steroids are commonly used, as well, but no controlled clinical trials assess the efficacy of this treatment.
DRESS is one of several terms that have been used to describe a severe idiosyncratic reaction to a drug that is characterized by a long latency of onset after exposure to the offending medication, a rash, involvement of internal organs, hematologic abnormalities, and systemic illness. Other synonymous names and acronyms include hypersensitivity syndrome (DIHS), anticonvulsant hypersensitivity syndrome, drug-induced hypersensitivity syndrome, drug-induced delayed multiorgan hypersensitivity syndrome, and drug-induced pseudolymphoma.
Signs and symptoms
The symptoms of DRESS syndrome usually begin several weeks after exposure to the offending drug. No gold standard exists for diagnosis, and at least two diagnostic criteria have been proposed. The RegiSCAR criteria  and the Japanese consensus group criteria are detailed in the table below.
|RegiSCAR inclusion criteria for DRESS syndrome. Three of the four starred criteria are required for diagnosis||Japanese consensus group diagnostic criteria for DIHS: Seven criteria are needed for diagnosis of DIHS or the first five criteria required for diagnosis of atypical DIHS.|
|Hospitalization||Maculopapular rash developing > 3 weeks after starting the suspected drug|
|Reaction suspected to be drug-related||Prolonged clinical symptoms 2 weeks after discontinuation of the suspected drug|
|Acute Rash*||Fever > 38 °C|
|Fever > 38 °C*||Liver abnormalities (ALT > 100 U/l or other organ involvement|
|Lymphadenopathy in at least two sites*||Leukocyte abnormalities|
|Involvement of at least one internal organ*||Leukocytosis ( > 11 x 109/l)|
|Blood count abnormalities (lymphopenia or lymphocytosis*, eosinophilia*, thrombocytopenia*)||Atypical lymphocytosis (>5%)|
|Human herpesvirus 6 reactivation|
Symptoms may be severe and involve many different organs. In a retrospective Taiwanese cohort study of 60 patients, these incidences were observed.
|Incidence of organ involvement in DRESS syndrome |
|Organ||Percent of patients with involvement|
|Incidence of hematologic abnormalities in DRESS syndrome |
|Abnormality||Percent of patients with abnormality|
Drugs that commonly induce DRESS syndrome include phenobarbital, carbamazepine, phenytoin, lamotrigine, minocycline, sulfonamides, allopurinol, modafinil, dapsone, ziprasidone, vancomycin, and most recently olanzapine. It has been associated with HHV-6 reactivation.
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- "Ziprasidone (Marketed as Geodon and Generics): Drug Safety Communication - Rare But Potentially Fatal Skin Reactions". 11 December 2014.
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- "Olanzapine: Drug Safety Communication - FDA Warns About Rare But Serious Skin Reactions". 10 May 2016.
- Volume 14, Issue 8, Pages 498-500 (December 2003)