ORPHA:636955
Endemic pemphigus foliaceus
Also known as: Fogo selvagem
Publications
564
83.6th percentile
Trials
0
Interventional, condition-specific
Researchers
900
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare autoimmune bullous skin disease characterized by the formation of subcorneal blisters mediated by autoantibodies against desmoglein 1, affecting predominantly seborrheic areas such as the head, neck, and upper trunk, but without any mucosal involvement. The lesions may be worsened by exposure to UV light and typically progress over weeks or months. The disease frequently occurs in children and young adults and is endemic to rural areas of Brazil and other countries in South America, as well as North Africa.
How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0043257
- MeSH:C535551
- UMLS:C0263314
Additional Mondo synonyms (1)
Brazilian pemphigus foliaceus
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.”
2/6 stages with a signal
No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
564 matched papers (276 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPartial
None under the specific name; 6 for broader category pemphigus foliaceus
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)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
564
564 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
564 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
276 in the last 10 years · high confidence · 83.6th percentile (publications denominator)
Phrase hits: 564 · MeSH hits: 1
Who's working on it?
900
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01Diaz LA34 papers · 2022
Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Papers in Europe PMC - 02Aoki V29 papers · 2025
Departamento de Dermatologia, Universidade de Sao Paulo, Brazil.
Papers in Europe PMC - 03
- 04Rivitti EA20 papers · 2020
Departamento de Dermatologia, Universidade de Sao Paulo, Brazil.
Papers in Europe PMC - 05Petzl-Erler ML15 papers · 2023
Laboratório de Genética Molecular Humana, Departamento de Genética, Universidade Federal do Paraná, Curitiba, Brazil.
Papers in Europe PMC - 06Abreu-Velez AM13 papers · 2022
Georgia Dermatopathology Associates, Atlanta, Georgia, USA. abreuvelez@yahoo.com
Papers in Europe PMC - 07Hans-Filho G13 papers · 2020
Department of Dermatology, Universidade Federal de Mato Grosso do Sul, Brazil.
Papers in Europe PMC - 08Qian Y11 papers · 2021
Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA. Electronic address: ye_qian@med.unc.edu.
Papers in Europe PMC - 09Li N10 papers · 2022
Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Papers in Europe PMC - 10Prisayanh P10 papers · 2022
Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
0
interventional trials for this specific condition
No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present). 6 trials are registered for pemphigus foliaceus, the broader category — shown separately because they may or may not enrol this specific subtype.
Data as of 27 July 2026
No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.
high confidence · 36.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
6 interventional trials matched pemphigus foliaceus, the broader category — listed below. Those studies are not counted in the condition-specific total.
Broader category: pemphigus foliaceus
6
Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.
Worth raising with a clinician. How we count trials.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This 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.
"Endemic pemphigus foliaceus" OR "Fogo selvagem" OR "Brazilian pemphigus foliaceus"
MeSH descriptor terms unioned into the query: Pemphigus and fogo selvagem
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Endemic pemphigus foliaceus" OR "Fogo selvagem" OR "Brazilian pemphigus foliaceus" OR "Pemphigus and fogo selvagem"
Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Parent-category trials query:
"pemphigus foliaceus"
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T19:29:40.226Z