DRESS Syndrome - Presentation

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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