schema: eds-research/corpus/v1
category:
  id: historiography
  label: Disease historiography
  description: "How the EDS concept formed: first descriptions, performer-era records, nosology revisions, and the modern classification era."
  order: 10
records:
  - id: hist-hippocratic-early-accounts
    kind: event
    title: Early descriptions of laxity and bruising attributed to antiquity
    summary: The standard historiography (Parapia and Jackson, 2008) traces descriptions consistent with joint laxity and easy bruising to Hippocratic-era medicine. These are retrospective attributions — the syndrome concept did not exist — and are indexed here as historical framing, not diagnosis.
    date: "-0400"
    date_precision: century
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: retrospective-account
        source_ids: [source-5c33d96b50581fbe82f8]
        note: Parapia and Jackson trace the earliest descriptions to Hippocrates, c. 400 BC.
    reviewed_at: "2026-09-16"
    tags: [retrospective-attribution]
  - id: hist-van-meekren-1682
    kind: event
    title: van Meek'ren reports extraordinary skin extensibility
    summary: Amsterdam surgeon Job Janszoon van Meek'ren documented a 23-year-old patient who could pull the skin of the chest over the head — commonly cited as the first detailed clinical description of the skin phenotype later associated with EDS.
    date: "1682"
    date_precision: year
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: primary-historical-document
        source_ids: [source-5c33d96b50581fbe82f8, source-3dd9471fb00916c6141b]
        note: Recorded through the historical-review literature; the original report is a 1682 Amsterdam surgical text.
    reviewed_at: "2026-09-16"
    tags: [retrospective-attribution, skin-phenotype]
  - id: hist-early-circus-performers
    kind: event
    title: "India Rubber Men and elastic performers in travelling shows"
    summary: Late nineteenth-century travelling shows featured performers whose extreme flexibility and skin elasticity were their livelihood — James Morris at Barnum and Bailey earned $150 per week in the 1880s. Medical literature of the era (Wile 1883, Gould and Pyle 1897) documented some of them. Retrospective attribution to EDS is plausible for some performers and unprovable for most; the record documents the social niche, not a diagnosis.
    date: "1883"
    date_precision: decade
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: contemporaneous-clinical-account
        source_ids: [source-5c33d96b50581fbe82f8]
        note: Wile 1883 and Gould and Pyle 1897 as cited by Parapia and Jackson.
    reviewed_at: "2026-09-16"
    tags: [folk-context, performers, retrospective-attribution]
  - id: hist-chernogubov-1892
    kind: event
    title: Chernogubov presents the first modern case reports
    summary: Nikolai Chernogubov (Tschernogobow) presented two patients to the Moscow Venereology and Dermatology Society with skin fragility and hyperelasticity, joint hypermobility and luxation, and molluscoid pseudotumours — the first comprehensive modern case description. The syndrome is still known as Chernogubov's syndrome in Russian literature.
    date: "1892"
    date_precision: year
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: primary-historical-document
        source_ids: [source-3dd9471fb00916c6141b]
      - stratum: historical
        tier: retrospective-account
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
    people: [Nikolai Chernogubov]
  - id: hist-ehlers-1901
    kind: event
    title: Edvard Ehlers recognizes the syndrome as a distinct entity
    summary: Danish dermatologist Edvard Lauritz Ehlers published a case report of a patient with joint laxity, stretchy skin, easy bruising, frequent knee subluxations, and delayed walking — the description for which the syndrome is named.
    date: "1901"
    date_precision: year
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: primary-historical-document
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
    people: [Edvard Ehlers]
  - id: hist-danlos-1908
    kind: event
    title: Henri-Alexandre Danlos fixes skin extensibility and fragility as cardinal features
    summary: French physician Henri-Alexandre Danlos, working with Pautier, described the skin features that completed the syndrome's early clinical picture. Decades later it emerged that Danlos's own case was likely pseudoxanthoma elasticum — an early lesson in the hazards of phenotype-only classification.
    date: "1908"
    date_precision: year
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: primary-historical-document
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
    people: [Henri-Alexandre Danlos]
  - id: hist-sack-1936-dysvascularis
    kind: event
    title: Sack describes status dysvascularis
    summary: Georg Sack described the vascular catastrophe form — arterial rupture and organ fragility — later formalized as the vascular type. This is the historical root of vEDS recognition.
    date: "1936"
    date_precision: year
    subtypes: [veds]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: retrospective-account
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
    people: [Georg Sack]
  - id: hist-named-ehlers-danlos-1949
    kind: event
    title: Johnson and Falls name the Ehlers-Danlos syndrome
    summary: The eponym joining Ehlers's and Danlos's names entered the literature in 1949, consolidating several separately described case families under one label.
    date: "1949"
    date_precision: year
    subtypes: [unspecified]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: retrospective-account
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
  - id: hist-barabas-1967-vascular
    kind: event
    title: Barabas establishes heterogeneity and the arterial complication profile
    summary: A. P. Barabas demonstrated that EDS was heterogeneous and delineated the form with arterial complications — the direct ancestor of the vascular subtype (type IV in Villefranche, vEDS today).
    date: "1967"
    date_precision: year
    subtypes: [veds]
    status: historical-record
    criteria_era: pre-nosology
    evidence:
      - stratum: historical
        tier: retrospective-account
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
    people: [A. P. Barabas]
  - id: hist-beighton-score-1973
    kind: event
    title: Beighton score published for epidemiological joint-mobility assessment
    summary: Beighton, Solomon, and Soskolne published the nine-point articular mobility score in an Annals of the Rheumatic Diseases population study — designed for epidemiology, later adopted as the clinical hypermobility measure still used in the 2017 hEDS criteria.
    date: "1973"
    date_precision: year
    subtypes: [all-eds]
    status: established
    criteria_era: pre-nosology
    evidence:
      - stratum: clinical
        tier: cohort-study
        source_ids: [source-8cddcecd9d7a698123e5]
    reviewed_at: "2026-09-16"
    people: [Peter Beighton]
  - id: hist-berlin-1988
    kind: event
    title: Berlin nosology expands EDS to eleven numbered types
    summary: The 1988 Berlin nosology for heritable connective-tissue disorders expanded EDS into eleven numbered types — a proliferation that blurred clinical boundaries and set up the later consolidation.
    date: "1988"
    date_precision: year
    subtypes: [all-eds]
    status: historical-record
    criteria_era: berlin-1988
    evidence:
      - stratum: historical
        tier: retrospective-account
        source_ids: [source-5c33d96b50581fbe82f8]
      - stratum: clinical
        tier: expert-review
        source_ids: [source-be972ed50decef4cbf0b]
    corroboration: convergent
    reviewed_at: "2026-09-16"
  - id: hist-villefranche-1997
    kind: event
    title: Villefranche nosology consolidates to six types
    summary: Beighton et al. published the revised Villefranche nosology (1997, printed 1998) consolidating EDS to six major types — classical (I/II), hypermobility (III), vascular (IV), kyphoscoliosis (VI), arthrochalasia (VIIA/B), dermatosparaxis (VIIC) — aligning clinical types with the emerging molecular era.
    date: "1998"
    date_precision: year
    subtypes: [all-eds]
    status: established
    criteria_era: villefranche-1997
    evidence:
      - stratum: clinical
        tier: consensus-statement
        source_ids: [source-a0e3bc5bd3a0b3545cf8]
    reviewed_at: "2026-09-16"
  - id: hist-international-2017
    kind: event
    title: 2017 International Classification defines thirteen subtypes
    summary: "Malfait et al. and the International EDS Consortium published the current classification: thirteen subtypes, molecular confirmation required for all except hEDS, a pathogenetic scheme grouping by pathway, and the companion framework separating hEDS from HSD. Management guidelines for comorbidities accompanied the criteria for the first time."
    date: "2017-03"
    date_precision: month
    subtypes: [all-eds]
    status: established
    criteria_era: international-2017
    evidence:
      - stratum: clinical
        tier: consensus-statement
        source_ids: [source-36ad1cab53c4cea4f4b4, source-d7dec285eaee780f18a3]
      - stratum: community
        tier: patient-org-synthesis
        source_ids: [source-c55aca51ea775bf6d160]
        note: The society's type pages present the same 13-type structure to patients.
    corroboration: convergent
    reviewed_at: "2026-09-16"
    people: [Fransiska Malfait]
    organizations: [International EDS Consortium, The Ehlers-Danlos Society]
  - id: hist-klk15-2025
    kind: event
    title: KLK15 identified as the first gene associated with hEDS
    summary: "The Norris Lab and colleagues published whole-exome work implicating kallikrein-family variants in hEDS: a recurrent KLK15 missense variant segregating in multiple families, burden enrichment across KLK genes, and a knock-in mouse recapitulating connective-tissue features. Published in iScience (August 2025) after a 2024 preprint and society announcement; hEDS remains a clinical diagnosis pending replication and HEDGE results."
    date: "2025-08"
    date_precision: month
    subtypes: [heds]
    status: emerging
    criteria_era: international-2017
    evidence:
      - stratum: clinical
        tier: mechanistic-study
        source_ids: [source-ee867f99ac8808a4ef80]
      - stratum: gray
        tier: preprint
        source_ids: [source-daaa8e28125b9b3bb775]
      - stratum: community
        tier: patient-org-synthesis
        source_ids: [source-bb268c000069896e8023]
      - stratum: registry
        tier: registry-report
        source_ids: [source-3440a2b9d5d99c580d3a]
    corroboration: convergent
    reviewed_at: "2026-09-16"
    reassess_by: "2026-12-16"
    organizations: [Norris Lab, Medical University of South Carolina, The Ehlers-Danlos Society]
    tags: [genetics, heds, frontier]