Drug reaction with eosinophilia and systemic symptoms

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Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) is a syndrome, caused by exposure to certain medications, that may cause a rash, fever, inflammation of internal organs, lymphadenopathy, and characteristic hematologic abnormalities such as eosinophilia, thrombocytopenia, and atypical lymphocytosis. The syndrome carries about a 10% mortality.[1] Treatment consists of stopping the offending medication and providing supportive care. Systemic steroids are commonly used as well; however, there are no controlled clinical trials to assess the efficacy of this treatment.[2]

The term was coined in a 1996 report in an attempt to simplify terminology for a syndrome recognized as early as 1959.[3][4]

Nomenclature

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 HSS (Hypersensitivity Syndrome), AHS (Anticonvulsant Hypersensitivity Syndrome), DIHS (Drug-Induced Hypersensitivity Syndrome), DIDMOHS (Drug-Induced Delayed Multiorgan Hypersensitivity Syndrome), and Drug-Induced Pseudolymphoma.[1]

Signs and symptoms

The symptoms of DRESS syndrome usually begin several weeks after exposure to the offending drug. There is no gold standard for diagnosis, and at least two diagnostic criteria have been proposed. The RegiSCAR criteria [5] and the Japanese consensus group criteria[6] are detailed in the table below.

RegiSCAR inclusion criteria for DRESS syndrome. Three of the four starred criteria required for diagnosis Japanese consensus group diagnostic criteria for DIHS. Seven criteria 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%)
Lymphadenopathy
Human herpesvirus 6 reactivation

Symptoms may be severe and involve many different organs. In a retrospective Taiwanese cohort study of 60 patients,[7] the following incidences were observed.

Incidence of organ involvement in DRESS syndrome [1]
Organ Percent of patients with involvement
Liver 80%
Kidney 40%
Pulmonary 33%
Cardiac/muscular 15%
Pancreas 5%
Incidence of hematologic abnormalities in DRESS syndrome [1]
Abnormality Percent of patients with abnormality
Atypical lymphocyte 63%
Eosinophilia 52%
Lymphocytopenia 45%
Thrombocytopenia 25%
Lymphocytosis 25%

Causes

Drugs that commonly induce DRESS syndrome include phenobarbital, carbamazepine, phenytoin,[8] lamotrigine, minocycline,[9] sulfonamides, allopurinol,[10] modafinil, dapsone, ziprasidone,[11] and most recently olanzapine.[12] It has been associated with HHV-6 reactivation.[13]

See also

References

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  13. ↑ Volume 14, Issue 8, Pages 498-500 (December 2003)

Further reading

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