Presentation
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 and the Japanese consensus group criteria 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,the following incidences were observed.
| Incidence of organ involvement in DRESS syndrome | |
|---|---|
| Organ | Percent of patients with involvement |
| Liver | 80% |
| Kidney | 40% |
| Pulmonary | 33% |
| Cardiac/muscular | 15% |
| Pancreas | 5% |
| Incidence of hematologic abnormalities in DRESS syndrome | |
|---|---|
| Abnormality | Percent of patients with abnormality |
| Atypical lymphocyte | 63% |
| Eosinophilia | 52% |
| Lymphocytopenia | 45% |
| Thrombocytopenia | 25% |
| Lymphocytosis | 25% |
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“He uses his folly like a stalking-horse, and under the presentation of that he shoots his wit.”
—William Shakespeare (1564–1616)