RARE DISEASERESEARCH ATLAS

ORPHA:50942

Striate palmoplantar keratoderma

high confidenceDisorder

Also known as: Keratosis palmoplantaris striata · Keratosis palmoplantaris striata et areata · Keratosis palmoplantaris varians of Wachters

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

Publications

183

56.2th percentile

Trials

0

Interventional, condition-specific

Researchers

970

Distinct authors in sample

Gene link

Readiness

1/6

Stages with a signal

Clinical definition (Orphanet)

Striate palmoplantar keratoderma is an isolated, focal, palmoplantar keratoderma characterized by linear hyperkeratosis along the flexor aspect of the fingers and on palms, as well as focal hyperkeratosis of the plantar skin. Patients present with painful thickening of the skin on palms and soles, with occasional fissuring, blistering and hyperhidrosis. Rarely, hyperkeratosis on other areas may be seen (knees, dorsal aspects of the digits). Histopatologically, widened intercellular spaces between keratinocytes are observed.

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.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (3)

keratosis palmoplantaris striata · keratosis palmoplantaris striata et areata · keratosis palmoplantaris varians of Wachters

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

    183 matched papers (58 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?

183

183 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.

183 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).

58 in the last 10 years · high confidence · 56.2th percentile (publications denominator)

Phrase hits: 183 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

970

Distinct author names in 183 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
    Green KJ16 papers · 2022

    Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611 Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611.

    Papers in Europe PMC
  2. 02
    Kelsell DP11 papers · 2019

    Centre for Cutaneous Research, Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, 4 Newark Street, London E1 2AT, UK. d.p.kelsell@qmul.ac.uk.

    Papers in Europe PMC
  3. 03
    Eady RA8 papers · 2009
    Papers in Europe PMC
  4. 04
    McGrath JA8 papers · 2013

    St John's Institute of Dermatology, King's College London (Guy's Campus), London, UK.

    Papers in Europe PMC
  5. 05
    Christiano AM7 papers · 2014

    Department of Genetics and Development, Columbia University, New York, New York 10032 Department of Dermatology, Columbia University, New York, New York 10032.

    Papers in Europe PMC
  6. 06
    Leigh IM7 papers · 2006
    Papers in Europe PMC
  7. 07
    Sprecher E6 papers · 2018

    Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

    Papers in Europe PMC
  8. 08
    Whittock NV6 papers · 2004

    Department of Cellular and Molecular Pathology, St John's Institute of Dermatology, The Guy's, King's College, and St Thomas' Hospitals' Medical School, London, UK. nwhittoc@hgmp.mrc.ac.uk

    Papers in Europe PMC
  9. 09
    Harmon RM5 papers · 2017

    Departments of Dermatology and Pathology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611.

    Papers in Europe PMC
  10. 10
    Amagai M4 papers · 2012

    Department of Dermatology, Keio University School of Medicine, Tokyo, Japan. amagai@sc.itc.keio.ac.jp

    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.

"Striate palmoplantar keratoderma" OR "Keratosis palmoplantaris striata" OR "Keratosis palmoplantaris striata et areata" OR "Keratosis palmoplantaris varians of Wachters" OR "Keratosis palmoplantaris varians of the Wachters"

Run this search on Europe PMC

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

"Striate palmoplantar keratoderma" OR "Keratosis palmoplantaris striata" OR "Keratosis palmoplantaris striata et areata" OR "Keratosis palmoplantaris varians of Wachters" OR "Keratosis palmoplantaris varians of the Wachters" OR "focal palmoplantar keratoderma" OR "hereditary palmoplantar keratoderma"

Recall-expansion terms: focal palmoplantar keratoderma, hereditary palmoplantar keratoderma

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 2026-07-27T00:45:16.515Z