Drug reaction with eosinophilia and systemic symptoms
Synonyms: DRESS syndrome · Drug rash with eosinophilia and systemic symptoms
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Drug reaction with eosinophilia and systemic symptoms | DRESS syndrome · Drug rash with eosinophilia and systemic symptoms |
| fr | Réaction médicamenteuse avec éosinophilie et symptômes systémiques | Eruption cutanée avec éosinophilie et symptômes systémiques · Hypersensibilité médicamenteuse avec éosinophiles et manifestations systémiques · Syndrome DRESS |
| de | Arzneimittelreaktion mit Eosinophilie und systemischen Symptomen | DRESS-Syndrom · Hypersensitivitäts-Syndrom mit Eosinophilie und systemischen Symptomen |
| es | Reacción a fármacos con eosinofilia y síntomas sistémicos | Síndrome DRESS · Erupción por fármacos con eosinofilia y síntomas sistémicos · Reacción a medicamentos con eosinofilia y síntomas sistémicos |
| it | Reazione ai farmaci con eosinofilia e sintomi sistemici | Eruzione da farmaco con eosinofilia e sintomi sistemici · Sindrome DRESS |
| nl | Reactie op medicatie met eosinofilie en systemische symptomen | DRESS-syndroom · Medicatiegeïnduceerde huiduitslag met eosinofilie en systemische symptomen |
| pt | Reação medicamentosa com eosinofilia e sintomas sistémicos | Exantema medicamentoso com eosinofilia e sintomas sistémicos · Síndrome DRESS |
| pl | Reakcja polekowa z eozynofilią i objawami ogólnoustrojowymi | Polekowa eozynofilia z objawami ogólnymi · Zespół DRESS |
| cs | Léky způsobená vyrážka s eozinofilií a systémovými symptomy | DRESS syndrom · Eozinofilní systémový syndrom způsobený reakcí na léčiva |
| uk | Медикаментозна реакція з еозинофілією та системними симптомами | DRESS-синдром · Медикаментозне висипання з еозинофілією та системними симптомами |
| zh | 伴嗜酸性粒细胞增多症和全身症状的药疹 | 药物反应嗜酸性粒细胞增多性系统性综合征 |
| tr | Eozinofili ve sistemik semptomlar ile seyreden ilaç döküntüsü | DRESS sendromu · İlaç reaksiyonu eozinofilik sistemik sendrom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | T88.7 | NTBT | attributed | validated |
| ICD-11 | EH65 | E | specific | validated |
| MONDO | 0015340 | E | validated | |
| UMLS | C3541994 | E | validated | |
| GARD | 13629 | E | validated | |
| MedDRA | 10073508 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:293815Toxic dermatosis
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
28 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0100665 | Angioedema | |
| HP:0001695 | Cardiac arrest | |
| HP:0010783 | Erythema | |
| HP:0001019 | Erythroderma | |
| HP:0001945 | Fever | |
| HP:0100326 | Immunologic hypersensitivity | |
| HP:0002383 | Infectious encephalitis | |
| HP:0002716 | Lymphadenopathy | |
| HP:0012733 | Macule | |
| HP:0000988 | Skin rash | |
| HP:0100646 | Thyroiditis | |
| HP:0001824 | Weight loss | |
| Frequent (79-30%) | ||
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0006515 | Interstitial pneumonitis | |
| HP:0100827 | Lymphocytosis | |
| HP:0002113 | Pulmonary infiltrates | |
| Occasional (29-5%) | ||
| HP:0006554 | Acute hepatic failure | |
| HP:0012735 | Cough | |
| HP:0002094 | Dyspnea | |
| HP:0030249 | Enanthema | |
| HP:0012115 | Hepatitis | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0012819 | Myocarditis | |
| HP:0000100 | Nephrotic syndrome | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0200039 | Pustule | |
| HP:0000083 | Renal insufficiency | |
| HP:0001970 | Tubulointerstitial nephritis | |
Run the differential on the 12 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/139402
- This record as JSON: /data/d/139402.json