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Start free with EleplanDrug reaction with eosinophilia and systemic symptoms
ORPHA:139402Disease
Also called DRESS syndrome · Drug rash with eosinophilia and systemic symptoms
What it is
A rare hypersensitivity reaction characterized by a generalized infiltrated skin rash with face edema, fever, enlarged lymph nodes, eosinophilia, lymphocytosis and more or less severe visceral involvement (e.g. hepatitis, nephritis, pneumonitis, myocarditis ect.) and, in some patients, reactivation of human herpes virus 6, Epstein-Barr virus and/or cytomegalovirus. Onset usually occurs 2-8 weeks after administration of the causal medication and is most frequently associated with anticonvulsants, antibacterial sulfonamides and allopurinol but many other medications have also been implicated. Histology is characterized by interface dermatitis, sometimes mixed with eczematous and an acute generalized exanthematous pustulosis-like pattern. Auto-immune sequelae may occur.
Key facts
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Very common80–99%
12Common30–79%
4Sometimes5–29%
12- Acute hepatic failure
- Cough
- Dyspnea
- Enanthema
- Hepatitis
- Increased total eosinophil count
- Myocarditis
- Nephrotic syndrome
and 4 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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