ORPHA:79099
Interstitial granulomatous dermatitis with arthritis
Also known as: Ackerman dermatitis syndrome · Ackerman syndrome · IGDA
Publications
322
71.4th percentile
Trials
0
Interventional, condition-specific
Researchers
1,008
Distinct authors in sample
Gene link
—
Readiness
1/6
Stages with a signal
Clinical definition (Orphanet)
Interstitial granulomatous dermatitis with arthritis is a rare rheumatologic disease characterized by the occurrence of inflammatory arthritis in association with large, erythematous, symmetrical cutaneous lesions (ranging from typical, but infrequent, cord-like lesions on the flanks to more common violaceous plaques on the trunk and limbs) featuring a typical histologic infiltrate mainly constituted of histiocytes.
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0019199
- UMLS:C4751206
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.”
1/6 stages with a signal
Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
322 matched papers (133 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 trialNot found
No matched interventional trial under our ClinicalTrials.gov rules
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?
322
322 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
322 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).
133 in the last 10 years · medium confidence · 71.4th percentile (publications denominator)
Phrase hits: 322 · MeSH hits: 0
Who's working on it?
1,008
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.
- 01Kimata H9 papers · 2005
Department of Allergy, Ujitakeda Hospital, 24-1, Umonji, UJi, Kyoto Prefecture 6110021, Japan. h-kimata@takedahp.or.jp
Papers in Europe PMC - 02Rogala B6 papers · 2008Papers in Europe PMC
- 03Goldbach-Mansky R5 papers · 2024
Translational Autoinflammatory Diseases Section, National Institutes of Health, Bethesda, Maryland, USA raphaela.goldbach-mansky@nih.gov fabrizio.debenedetti@opbg.net rebecca.marsh@cchmc.org cannas@chop.edu.
Papers in Europe PMC - 04Kasperska-Zajac A5 papers · 2008
Clinical Department of Internal Diseases, Allergology and Clinical Immunology, Medical University of Silesia, Zabrze, Poland. kasperska@plusnet.pl
Papers in Europe PMC - 05Brzoza Z4 papers · 2008
Katedra i Klinika Chorób Wewnetrznych, Alergologii i Immunologii Klinicznej SAM w Zabrzu.
Papers in Europe PMC - 06Ebstein F4 papers · 2025
Institute of Medical Biochemistry and Molecular Biology, University Medicine Greifswald, 17475 Greifswald, Germany.
Papers in Europe PMC - 07de Jesus AA3 papers · 2024
Translational Autoinflammatory Diseases Section, LCIM, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Papers in Europe PMC - 08Fuiano N3 papers · 2011
Servizio di Pediatria Ambulatoriale A. USL FG1, San Severo, Foggia. fuiano50@tin.it
Papers in Europe PMC - 09Haahtela T3 papers · 2005Papers in Europe PMC
- 10Henry T3 papers · 2026
CIRI, Centre International de Recherche en Infectiologie, Univ Lyon, Inserm U1111, Université Claude Bernard Lyon 1, CNRS, UMR5308, ENS de Lyon, F-69007, LYON, France. thomas.henry@inserm.fr.
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).
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.
medium 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.
See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.
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.
"Interstitial granulomatous dermatitis with arthritis" OR "Ackerman dermatitis syndrome" OR "Ackerman syndrome"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Interstitial granulomatous dermatitis with arthritis" OR "Ackerman dermatitis syndrome" OR "Ackerman syndrome"
Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: IGDA
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- "Ackerman syndrome" also appears on ORPHA:2561
Ingested 2026-07-27T01:56:42.093Z