RARE DISEASERESEARCH ATLAS

ORPHA:178540

Primary cutaneous follicle center lymphoma

high confidenceDisorder

Also known as: PCFCL

Publications

531

86.8th percentile

Trials

2

Interventional, condition-specific

Researchers

1,047

Distinct authors in sample

Gene link

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare, indolent primary cutaneous B-cell lymphoma characterized by a solitary or grouped erythematous plaques or tumors, preferentially located on the head, neck or trunk region, and composed of centroblasts and centrocytes arranged in a follicular, diffuse, or mixed growth pattern. The lesions are smooth and typically do not ulcerate. The neoplastic cells express pan B cell markers and Bcl-6, and typically lack Bcl-2.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

Crosti's disease · Reticulohistiocytoma of the dorsum · cutaneous follicle center lymphoma · cutaneous follicle centre lymphoma

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

    531 matched papers (352 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

    2 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?

531

531 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=5449) is 41.

531 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).

352 in the last 10 years · high confidence · 86.8th percentile (publications denominator)

Phrase hits: 531 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,047

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
    Hristov AC7 papers · 2025

    From the Department of Pathology, University of Michigan Medical Center, Ann Arbor (Drs Skala and A. C. Hristov); the Department of Internal Medicine, Section of Radiation Oncology, Wright Patterson Air Force Base (Dr B. Hristov); and the Department of Dermatology, University of Michigan Medical Center (Dr A. C. Hristov).

    Papers in Europe PMC
  2. 02
    Wilcox RA7 papers · 2025

    Division of Hematology/Oncology University of Michigan Cancer Center, 1500 E. Medical Center Drive Room 4310 CC, Ann Arbor, Michigan, 48109-5948. rywilcox@med.umich.edu.

    Papers in Europe PMC
  3. 03
    Alaibac M6 papers · 2024

    Unit of Dermatology, University of Padua, Padua, Italy.

    Papers in Europe PMC
  4. 04
    Willemze R6 papers · 2025

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

    Papers in Europe PMC
  5. 05
    Cerroni L5 papers · 2023

    Department of Dermatology, Medical University of Graz, Graz, Austria.

    Papers in Europe PMC
  6. 06
    Grandi V5 papers · 2026

    Department of Human Health Sciences, Section of Dermatology, Piero Palagi Hospital, Florence, Italy.

    Papers in Europe PMC
  7. 07
    Jansen PM5 papers · 2025

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

    Papers in Europe PMC
  8. 08
    Pimpinelli N5 papers · 2026

    Department of Health Sciences, Division of Dermatology, University of Florence, Florence, Italy.

    Papers in Europe PMC
  9. 09
    Tejasvi T5 papers · 2025

    Department of Dermatology, University of Michigan, Ann Arbor, Michigan, USA.

    Papers in Europe PMC
  10. 10
    Vermeer MH5 papers · 2025

    Dermatology.

    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; 1 currently recruiting in our sample.

Data as of 27 July 2026

2 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 82.4th percentile).

high confidence · 82.4th 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.

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 follicle center lymphoma" OR "PCFCL" OR "Crosti's disease" OR "Reticulohistiocytoma of the dorsum" OR "Reticulohistiocytoma of dorsum" OR "cutaneous follicle center lymphoma" OR "cutaneous follicle centre lymphoma"

Run this search on Europe PMC

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

"Primary cutaneous follicle center lymphoma" OR "PCFCL" OR "Crosti's disease" OR "Reticulohistiocytoma of the dorsum" OR "Reticulohistiocytoma of dorsum" OR "cutaneous follicle center lymphoma" OR "cutaneous follicle centre lymphoma"

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 · 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

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 2026-07-27T08:55:31.239Z