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ORPHA:178544

Primary cutaneous diffuse large B-cell lymphoma, leg type

high confidenceDisorder

Also known as: PCDLBCL,LT

Publications

368

82.8th percentile

Trials

6

Interventional, condition-specific

Researchers

1,121

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare, aggressive, primary cutaneous B-cell lymphoma characterized by rapidly , red to bluish, often ulcerating, nodular tumors predominantly involving the lower legs. Histology shows sheets of centroblasts and immunoblasts that spare the epidermis, but infiltrate the dermis and subcutaneous tissues, and often disseminate extracutaneously. The neoplastic cells typically express CD20, CD79a, Bcl-2, MUM-1, and FOXP1, but are negative for CD10.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (1)

primary cutaneous diffuse large B-cell lymphoma, Leg type

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

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

    368 matched papers (265 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    6 matched on ClinicalTrials.gov (1 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)

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?

368

368 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.

368 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).

265 in the last 10 years · high confidence · 82.8th percentile (publications denominator)

Phrase hits: 368 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,121

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
    Beylot-Barry M9 papers · 2026

    Service de Dermatologie, CHU de Bordeaux, Bordeaux, France; INSERM U1053, Equipe Oncogenèse des lymphomes cutanés, Université de Bordeaux.

    Papers in Europe PMC
  2. 02
    Willemze R9 papers · 2025

    Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.

    Papers in Europe PMC
  3. 03
    Jansen PM8 papers · 2025

    Department of Pathology, Leiden University Medical Center, L1-Q, P.O. box 9600, 2300RC, Leiden, The Netherlands.

    Papers in Europe PMC
  4. 04
    Wilcox RA7 papers · 2025

    Division of Hematology/Oncology, University of Michigan Cancer Center, Ann Arbor, Michigan.

    Papers in Europe PMC
  5. 05
    Alaibac M6 papers · 2024

    Dermatologic Clinic, University of Padova, Padova, Italy.

    Papers in Europe PMC
  6. 06
    Merlio JP6 papers · 2026

    INSERM U1053, Equipe Oncogenèse des lymphomes cutanés, Université de Bordeaux; Service de Biologie des tumeurs, CHU de Bordeaux, Pessac, France.

    Papers in Europe PMC
  7. 07
    Pham-Ledard A6 papers · 2026

    Service de Dermatologie, CHU de Bordeaux, Bordeaux, France; INSERM U1053, Equipe Oncogenèse des lymphomes cutanés, Université de Bordeaux.

    Papers in Europe PMC
  8. 08
    Vermaat JSP6 papers · 2025

    Department of Hematology, Leiden University Medical Center, Leiden, The Netherlands.

    Papers in Europe PMC
  9. 09
    Vermeer MH6 papers · 2025

    Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.

    Papers in Europe PMC
  10. 10
    Berti E5 papers · 2022

    Unit of Dermatology, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico Milan, 20122, Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

6

interventional trials for this specific condition

6 interventional trials matched this specific condition name; 1 currently recruiting in our sample. 1,017 trials are registered for diffuse large B-cell lymphoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

6 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 89th percentile).

high confidence · 89th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: diffuse large B-cell lymphoma

1,017

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

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 on the overview but are not added to the query string.

"Primary cutaneous diffuse large B-cell lymphoma, leg type" OR "PCDLBCL,LT"

Run this search on Europe PMC

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

"Primary cutaneous diffuse large B-cell lymphoma, leg type" OR "PCDLBCL,LT"

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: 6 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"diffuse large B-cell lymphoma"

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 2026-07-27T08:56:02.506Z