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ORPHA:1656

Dermatitis herpetiformis

high confidenceDisorder

Also known as: Duhring-Brocq disease

Publications

7,126

90.6th percentile

Trials

2

Interventional, condition-specific

Researchers

948

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. It may also appear as a consequence of gluten intolerance.

How rare: 1-5 / 10 000 — about one to five people per ten thousand (still uncommon, but less ultra-rare).

Orphanet entry

Cross-references

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

Additional Mondo synonyms (3)

Duhring's disease · Durhing-Brocq disease · dermatitis herpetiformis

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.”

4/6 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

    7,126 matched papers (1,998 in last 10 years) Source

  3. Phenotype characterisedPresent

    27 HPO annotations (e.g. Abnormality of the thyroid gland; Edema; Pruritus) Source

  4. Animal modelNot found

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

  5. Orphan designationPresent

    1 FDA designation (1 FDA orphan-indication approval) — e.g. Sulfapyridine Source

  6. Interventional trialPresent

    2 matched on ClinicalTrials.gov

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)

27

Associated phenotypes · MONDO:0015614

  • Abnormality of the thyroid gland
  • Edema
  • Pruritus
  • Lichenification
  • Abnormality of the nervous system

Showing 5 of 27 — 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)

1

Designation · 1 with FDA orphan-indication approval

  • FDA SulfapyridineDermatitis Herpetiformis · 1990-09-10 · Not FDA Approved for Orphan Indication

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

8

Drugs / clinical candidates · MONDO_0015614

CTD chemicals (MyDisease.info)

1 associated chemical. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Dapsone · therapeutic

MyDisease.info · MONDO:0015614

Literature

Is anyone studying this?

7,126

7,126 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.

7,126 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).

1,998 in the last 10 years · high confidence · 90.6th percentile (publications denominator)

Phrase hits: 7,126 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

948

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
    Salmi T10 papers · 2026

    Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  2. 02
    Kaukinen K9 papers · 2026

    Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  3. 03
    Hervonen K8 papers · 2026

    Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  4. 04
    Huhtala H7 papers · 2026

    Faculty of Social Sciences, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  5. 05
    Pasternack C6 papers · 2026

    Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  6. 06
    Reunala T5 papers · 2026

    Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  7. 07
    Schmidt E5 papers · 2026

    Department of Dermatology.

    Papers in Europe PMC
  8. 08
    Drenovska K4 papers · 2026

    Department of Dermatology and Venereology, Medical University - Sofia, Sofia , Bulgaria.

    Papers in Europe PMC
  9. 09
    Lindfors K4 papers · 2026

    Celiac Disease Research Center, Tampere University, Tampere, Finland.

    Papers in Europe PMC
  10. 10
    Caproni M3 papers · 2026

    Deparment of Health Sciences, Section of Dermatology, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Florence , Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

2

interventional trials for this specific condition

2 interventional trials matched this specific condition name; none in our sample are currently recruiting. 1,584 trials are registered for dermatitis, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

2 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 84.5th percentile).

high confidence · 84.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Broader category: dermatitis

1,584

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.

Recruiting under the broader category

Observational and natural-history studies

3 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 5 · after dedupe 5 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 5 · dropped 0 · fetched 2026-07-29

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 (5)

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 Dermatitis herpetiformis — 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.

"Dermatitis herpetiformis" OR "Duhring-Brocq disease" OR "Duhring's disease" OR "Durhing-Brocq disease"

Run this search on Europe PMC

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

"Dermatitis herpetiformis" OR "Duhring-Brocq disease" OR "Duhring's disease" OR "Durhing-Brocq disease"

Interventional trials matched via: 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: 2 interventional · 3 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"dermatitis"

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

Ingested 2026-07-26T17:53:22.503Z