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 attributions made in hindsight: the syndrome had not been described, and no one in these accounts was diagnosed with it." 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. The report is 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: retrospective-account source_ids: [source-5c33d96b50581fbe82f8] 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, who performed with 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. Attributing EDS to them in hindsight is plausible for some performers and unprovable for most. date: "1883" date_precision: decade subtypes: [unspecified] status: historical-record criteria_era: pre-nosology evidence: - stratum: historical tier: retrospective-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 description: In 1892 Nikolai Chernogubov presented two patients with fragile, stretchy skin and loose joints to a Moscow medical society. 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. It is 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-fd495bad5265f19454c2] - stratum: historical tier: retrospective-account source_ids: [source-5c33d96b50581fbe82f8] - stratum: clinical tier: expert-review source_ids: [source-be972ed50decef4cbf0b] corroboration: single-origin reviewed_at: "2026-09-16" people: [Nikolai Chernogubov] - id: hist-ehlers-1901 kind: event title: Edvard Ehlers recognizes the syndrome as a distinct entity description: In 1901 Danish dermatologist Edvard Ehlers reported a patient with loose joints, stretchy skin, easy bruising, and knee subluxations. 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 syndrome takes part of its name from this description. date: "1901" 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: single-origin 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. date: "1908" 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: single-origin 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 a form with arterial rupture and organ fragility that was 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] 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: refuted criteria_era: pre-nosology evidence: - stratum: historical tier: retrospective-account source_ids: [source-5c33d96b50581fbe82f8] - stratum: clinical tier: expert-review source_ids: [source-be972ed50decef4cbf0b] note: The chapter credits Johnson and Falls (1949) with the first report of autosomal-dominant inheritance, in a family with 32 affected members, and cites Ronchese's 1936 paper on “the so-called Ehlers-Danlos syndrome”. corroboration: refuted reviewed_at: "2026-09-16" - id: hist-barabas-1967-vascular kind: event title: Barabas establishes heterogeneity and the arterial complication profile description: A. P. Barabas showed in 1967 that EDS is more than one disorder and described the form with arterial complications, the forerunner of vascular EDS. 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] 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 description: Beighton, Solomon, and Soskolne published the nine-point Beighton score in 1973. The 2017 hEDS criteria use it to measure joint hypermobility. summary: Beighton, Solomon, and Soskolne published the nine-point articular mobility score in 1973, in an Annals of the Rheumatic Diseases study of joint mobility in an African population. It was designed for epidemiology and later became the clinical measure of hypermobility used in the 2017 hEDS criteria. date: "1973" date_precision: year subtypes: [all-eds] status: established criteria_era: pre-nosology evidence: - stratum: clinical tier: cross-sectional-study source_ids: [source-0bcf3a495f5d5a3f3899] - stratum: clinical tier: consensus-statement source_ids: [source-36ad1cab53c4cea4f4b4] note: The 2017 hEDS criteria assess generalized joint hypermobility with the Beighton score. 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. The proliferation 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] note: This chapter says nine types of EDS were proposed in the 1988 nosology. corroboration: contested reviewed_at: "2026-09-16" - id: hist-villefranche-1997 kind: event title: Villefranche nosology consolidates to six types description: The 1997 Villefranche nosology, published in 1998, consolidated EDS into six major types. summary: "Beighton et al. published the revised Villefranche nosology (1997, printed 1998), consolidating EDS into six major types: classical (I/II), hypermobility (III), vascular (IV), kyphoscoliosis (VI), arthrochalasia (VIIA/B), and dermatosparaxis (VIIC). The revision aligned 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 description: The 2017 International Classification defines thirteen EDS types and requires genetic confirmation for all except hEDS. 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: single-origin reviewed_at: "2026-09-16" people: [Fransiska Malfait] organizations: [International EDS Consortium, The Ehlers-Danlos Society] - id: hist-klk15-2025 kind: event title: "2025: KLK15 published as a candidate gene for hEDS" description: In August 2025 the Norris Lab published a KLK15 variant as a candidate cause of hEDS. hEDS remains a clinical diagnosis. summary: "In August 2025 the Norris Lab and colleagues published whole-exome work in iScience implicating kallikrein (KLK) gene variants in hEDS: a recurrent KLK15 variant segregating in multiple families, rare and low-frequency variants in 14 of the 15 KLK genes, and a knock-in mouse with hEDS features in tendons and heart valves. A preprint and The Ehlers-Danlos Society's summary appeared in June 2024. The society says hEDS remains a clinical diagnosis and that replication, including in the HEDGE study, is needed." 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: single-origin 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]