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ORPHA:139402

Drug reaction with eosinophilia and systemic symptoms

low confidenceDisorder

Also known as: DRESS syndrome · Drug rash with eosinophilia and systemic symptoms

Publications

47,897

Trials

4

Interventional, condition-specific

Researchers

1,056

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

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.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (6)

DHS · DRESS · Drug hypersensitivity syndrome · Drug reaction with eosinophilia and Systemic symptoms · dress · drug reaction eosinophilic systemic syndrome

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

3/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    47,897 matched papers (19,760 in last 10 years) Source

  3. Phenotype characterisedPresent

    28 HPO annotations (e.g. Skin rash; Fever; Pulmonary infiltrates) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    4 matched on ClinicalTrials.gov (2 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

28

Associated phenotypes · MONDO:0015340

  • Skin rash
  • Fever
  • Pulmonary infiltrates
  • Elevated circulating hepatic transaminase concentration
  • Interstitial pneumonitis

Showing 5 of 28 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

6 associated chemicals · 96 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Carbamazepine · marker/mechanism
  • Ciprofloxacin · marker/mechanism
  • Phenytoin · marker/mechanism
  • Rifampin · marker/mechanism
  • Vancomycin · marker/mechanism
  • voxelotor · marker/mechanism

Pathways: NF-kappa B signaling pathway; PI3K-Akt signaling pathway; Focal adhesion; ECM-receptor interaction; Complement and coagulation cascades; Platelet activation; Toll-like receptor signaling pathway; Prion diseases

MyDisease.info · MONDO:0015340

Literature

Is anyone studying this?

47,897

47,897 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

47,897 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

19,760 in the last 10 years · low confidence

Phrase hits: 47,897 · MeSH hits: 23

Open Europe PMC search

Who's working on it?

1,056

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Liu X4 papers · 2026

    Department of Geriatrics, Peking University First Hospital, Beijing, People's Republic of China.

    Papers in Europe PMC
  2. 02
    Agrebi M3 papers · 2026

    Pharmacology, Pharmacovigilance Center of Sousse, Faculty of Medicine of Sousse, University of Sousse, Sousse, TUN.

    Papers in Europe PMC
  3. 03
    Ben Salem C3 papers · 2026

    Pharmacology, Pharmacovigilance Center of Sousse, Faculty of Medicine of Sousse, University of Sousse, Sousse, TUN.

    Papers in Europe PMC
  4. 04
    Kalfoutzou A3 papers · 2026

    Department of Medical Oncology, 251 Air Force General Hospital, Athens, GRC.

    Papers in Europe PMC
  5. 05
    Mockenhaupt M3 papers · 2026

    Dokumentationszentrum schwerer Hautreaktionen (dZh), Department of Dermatology, Medical Center, University of Freiburg, Freiburg, Germany.

    Papers in Europe PMC
  6. 06
    Mylonakis A3 papers · 2026

    First Department of Surgery, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, GRC.

    Papers in Europe PMC
  7. 07
    Peter J3 papers · 2026

    Division of Allergology and Clinical Immunology, Department of Medicine, University of Cape Town, Cape Town, South Africa.

    Papers in Europe PMC
  8. 08
    Phillips E3 papers · 2026

    Department of Medicine, Vanderbilt Medical Centre, Nashville, Tennessee.

    Papers in Europe PMC
  9. 09
    Piperis C3 papers · 2026

    Department of Cardiology, Gennimatas General Hospital, Athens, GRC.

    Papers in Europe PMC
  10. 10
    Sahnoun D3 papers · 2026

    Pharmacology, Pharmacovigilance Center of Sousse, Faculty of Medicine of Sousse, University of Sousse, Sousse, TUN.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

4

interventional trials for this specific condition

4 interventional trials matched this specific condition name; 2 currently recruiting in our sample.

Data as of 11 September 2026

4 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 88.1th percentile).

low confidence · 88.1th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

4 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

4 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 9 · after dedupe 9 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 9 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (9)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Drug reaction with eosinophilia and systemic symptoms — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Drug reaction with eosinophilia and systemic symptoms" OR "DRESS syndrome" OR "Drug rash with eosinophilia and systemic symptoms" OR "DRESS" OR "Drug hypersensitivity syndrome" OR "drug reaction eosinophilic systemic syndrome"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Drug Hypersensitivity Syndrome

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Drug reaction with eosinophilia and systemic symptoms" OR "DRESS syndrome" OR "Drug rash with eosinophilia and systemic symptoms" OR "DRESS" OR "Drug hypersensitivity syndrome" OR "drug reaction eosinophilic systemic syndrome"

Interventional trials matched via: both, phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 4 interventional · 4 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: DHS

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (47897) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T07:34:19.289Z