RARE DISEASERESEARCH ATLAS

ORPHA:79479

Pemphigus vegetans

high confidence

Clinical definition (Orphanet)

A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3.

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.

Orphanet entry

Is anyone studying this?

739

739 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=183) is 38.

739 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 38 (publications denominator n=183).

305 in the last 10 years · high confidence · 85.5th percentile (publications denominator)

Is a treatment being tested?

0

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). 55 trials are registered for pemphigus, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 26 July 2026

55

trials for pemphigus, the broader category this belongs to

Trials registered for a broader category may or may not enrol people with this specific subtype — eligibility criteria vary, and the trial record often doesn't say. Worth raising with a clinician. How we count trials.

No matched interventional trials. This is true for 59.2% of diseases in the trials denominator (151 of 255). Here are the researchers publishing on it.

high confidence · 29.6th percentile (trials denominator)

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.

Who's working on it?

917

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.

  1. 01
    Ishii N9 papers · 2024

    Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.

    Papers in Europe PMC
  2. 02
    Hashimoto T7 papers · 2024

    Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.

    Papers in Europe PMC
  3. 03
    De D5 papers · 2026

    Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

    Papers in Europe PMC
  4. 04
    Koga H4 papers · 2025

    Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.

    Papers in Europe PMC
  5. 05
    Behera B3 papers · 2026

    Department of Dermatology and STD, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, India.

    Papers in Europe PMC
  6. 06
    Daneshpazhooh M3 papers · 2025

    Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

    Papers in Europe PMC
  7. 07
    Handa S3 papers · 2025

    Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

    Papers in Europe PMC
  8. 08
    Mahajan R3 papers · 2025

    Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

    Papers in Europe PMC
  9. 09
    Murrell DF3 papers · 2025

    Department of Dermatology, St. George Hospital, Sydney, NSW, Australia.

    Papers in Europe PMC
  10. 10
    Singh S3 papers · 2025

    Department of Dermatology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr RML Hospital, New Delhi, India.

    Papers in Europe PMC

Recruiting interventional trials

Trials testing a treatment from the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

55 interventional trials matched pemphigus, the broader category — see the summary above. Those studies are not counted in the condition-specific total.

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 2026-07-26

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 below but are not added to the query string.

"Pemphigus vegetans"

Run this search on Europe PMC

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

"Pemphigus vegetans"

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

Cross-references (from Mondo): UMLS:C0263316

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

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

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