RARE DISEASERESEARCH ATLAS

ORPHA:178522

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma

high confidenceDisorder

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

36

27.5th percentile

Trials

0

Interventional, condition-specific

Researchers

168

Distinct authors in sample

Gene link

Readiness

1/6

Stages with a signal

Clinical definition (Orphanet)

A rare, primary cutaneous T-cell lymphoma characterized by solitary cutaneous nodule or only regional disease, typically occurring on the head and neck, and involving entire dermis. Sometimes, subcutis and adnexal structures are involved, as well. The infiltrate is nodular or diffuse, composed of small to medium sized pleomorphic lymphocytes and showing mild to moderate cytologic atypia. Neoplastic T-cells are mixed with B-cells, histiocytes, plasma cells and eosinophils.

Orphanet entry

Cross-references

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

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.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    36 matched papers (10 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 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?

36

36 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.

36 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).

10 in the last 10 years · high confidence · 27.5th percentile (publications denominator)

Phrase hits: 36 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

168

Distinct author names in 36 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
    Magro CM5 papers · 2025

    Dermatologist, Department of Dermatopathology, Weill-Cornell Medical College and New York Presbyterian Hospital, New York, NY.

    Papers in Europe PMC
  2. 02
    Baum CL2 papers · 2011

    Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa.

    Papers in Europe PMC
  3. 03
    Cerroni L2 papers · 2009

    Department of Dermatology, Medical University of Graz, Austria. lorenzo.cerroni@meduni-graz.at

    Papers in Europe PMC
  4. 04
    Li X2 papers · 2025

    Pathologist, Department of Pathology, Montefiore Medical Center, The University Hospital of Albert Einstein College of Medicine, Bronx, NY; and.

    Papers in Europe PMC
  5. 05
    Liu V2 papers · 2011

    Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa; Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa. Electronic address: vincent-liu@uiowa.edu.

    Papers in Europe PMC
  6. 06
    Myskowski PL2 papers · 2017

    Department of Medicine, Dermatology Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

    Papers in Europe PMC
  7. 07
    Swick BL2 papers · 2011

    Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa; Department of Pathology, University of Iowa Hospitals and Clinics, Iowa City, Iowa.

    Papers in Europe PMC
  8. 08
    Trautinger F2 papers · 2016

    Department of Dermatology and Venereology, Karl Landsteiner University of Health Sciences, St. Pölten, Austria.

    Papers in Europe PMC
  9. 09
    Ajjamada L1 paper · 2023

    Division of Hematology, Department of Medicine, Jewish General Hospital, McGill University, Montréal, QC H3T 1E2, Canada.

    Papers in Europe PMC
  10. 10
    Alberti-Violetti S1 paper · 2016

    UOC Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.

    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.

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.

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 2026-07-26

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 CD4+ small/medium-sized pleomorphic T-cell lymphoma"

Run this search on Europe PMC

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

"Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma" OR "indolent primary cutaneous T-cell lymphoma"

Recall-expansion terms: indolent primary cutaneous T-cell lymphoma

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

Query health: suspect — strategies attempted: phrase, recall-expansion; 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 2026-07-27T08:54:28.959Z