{"schema":"https://ver.cy/schemas/card/1.0.0","id":"vr.tr.degos-disease","code":"thing-q911136","url":"https://ver.cy/models/thing/q911136/","name":"Degos disease","alternateNames":[],"kind":"thing","status":"published","version":"0.1.0-research.1","language":"en","classifiers":{"family":"Thing Registry","category":"Cross-cutting context","entryKind":"thing","plane":"XCT","domain":["XCT.STA"],"industry":[],"navPath":"","tags":[],"facets":{}},"whatItIs":"Degos disease, also called atrophic papulosis, is a rare occlusive vasculopathy characterized by papules that develop porcelain-white atrophic centres with erythematous rims, with disease either confined to the skin or involving internal organs, particularly the gastrointestinal tract and central nervous system.","purpose":"Enable an agent to recognise evidence for Degos disease, distinguish cutaneous-only observations from systemic involvement, and support clinician-reviewed assessment and follow-up.","scope":{"in":["Disease names, definition boundaries and versioned terminology mappings","Characteristic skin lesions and clinicopathological diagnostic evidence","Cutaneous-only and systemic involvement assessed over time","Vascular mechanisms, associated conditions and population evidence","Surveillance, escalation triggers and treatment evidence"],"out":["The patient's complete medical record and personal identity","Individual consultations, admissions and billing transactions","Dowling-Degos disease as a separate pigmentary disorder","Independent models of lupus, antiphospholipid syndrome and other differential diagnoses","Drug product specifications and prescribing protocols","Procedural models for biopsy, imaging or bowel surgery"],"boundaries":[]},"distinguishingFeatures":["Look for papules developing depressed porcelain-white centres with an erythematous rim; record morphology and evolution rather than treating any white scar as diagnostic. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)","Seek agreement between the clinical pattern and skin-biopsy evidence of small-vessel occlusion; there is no specific diagnostic blood test. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)","Distinguish characteristic skin disease alone from accompanying gastrointestinal or central nervous system involvement, which changes assessment of systemic disease. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)","Exclude name-based confusion with Dowling-Degos disease, whose defining presentation is pigmentation particularly in skin folds. [DermNet](https://dermnetnz.org/topics/dowling-degos-disease)"],"structure":{"bundles":[{"id":"identity-and-diagnostic-boundaries","name":"Identity and diagnostic boundaries","description":"Establish what Degos disease denotes and what evidence supports applying the name.","layers":[{"id":"names-and-classification","name":"Names and classification","description":"Connect the registry identity to clinical terminology without silently equating labels with prognosis.","findings":[{"id":"name-and-code-equivalence","name":"Name and code equivalence","description":"Record source-specific use of Degos disease, Köhlmeier-Degos disease and malignant atrophic papulosis, including whether each label covers cutaneous-only disease.","questions":[{"text":"Does the cited terminology use malignant atrophic papulosis for the full disease spectrum or specifically for systemic disease?","id":"name-and-code-equivalence-q01","kind":"definition"},{"text":"Which ICD-10, ICD-11 and SNOMED CT mappings are verified in a named release, and which are broader than Degos disease?","id":"name-and-code-equivalence-q02","kind":"provenance"}]}]},{"id":"diagnostic-justification","name":"Diagnostic justification","description":"Expose the reasoning and alternatives behind a diagnostic assertion.","findings":[{"id":"criteria-and-mimics","name":"Criteria and mimics","description":"Require an attributed diagnostic framework and an explicit distinction between Degos disease and a Degos-like lesion pattern.","questions":[{"text":"Which published criteria or expert assessment support the diagnosis, and what issuing body, publication date or revision applies?","id":"criteria-and-mimics-q01","kind":"provenance"},{"text":"How were Dowling-Degos disease, lupus-associated lesions and other occlusive vasculopathies assessed as alternatives?","id":"criteria-and-mimics-q02","kind":"boundary"}]}]}]},{"id":"cutaneous-and-tissue-evidence","name":"Cutaneous and tissue evidence","description":"Represent lesion appearance, evolution and biopsy interpretation together.","layers":[{"id":"lesion-evolution","name":"Lesion evolution","description":"Track the observable development and distribution of individual and grouped lesions.","findings":[{"id":"documented-lesion-pattern","name":"Documented lesion pattern","description":"Retain dated descriptions or photographs and distinguish newly appearing lesions from residual atrophic marks.","questions":[{"text":"Which lesions show a depressed porcelain-white centre and surrounding erythema, and where are they distributed?","id":"documented-lesion-pattern-q01","kind":"measurement"},{"text":"How many new lesions appeared during the stated interval, and which observations document their evolution?","id":"documented-lesion-pattern-q02","kind":"measurement"}]}]},{"id":"biopsy-correlation","name":"Biopsy correlation","description":"Link histology to the sampled lesion and the clinical interpretation.","findings":[{"id":"sample-specific-support","name":"Sample-specific support","description":"Capture reported vascular occlusion and tissue injury without treating an isolated pathology phrase as sufficient diagnosis.","questions":[{"text":"Which vascular and tissue changes did the pathologist report, and what lesion age and sampling depth were documented?","id":"sample-specific-support-q01","kind":"provenance"},{"text":"Does the report support Degos disease, remain nonspecific or favour an alternative when correlated with the clinical findings?","id":"sample-specific-support-q02","kind":"boundary"}]}]}]},{"id":"systemic-extent-and-course","name":"Systemic extent and course","description":"Track internal-organ evidence and changes in disease extent over time.","layers":[{"id":"organ-specific-evidence","name":"Organ-specific evidence","description":"Represent symptoms, investigation results and attribution separately for each organ.","findings":[{"id":"internal-involvement-assessment","name":"Internal involvement assessment","description":"Prioritise gastrointestinal and neurological assessment while allowing other organ findings and competing explanations.","questions":[{"text":"What evidence supports or argues against gastrointestinal or central nervous system involvement, and when was it obtained?","id":"internal-involvement-assessment-q01","kind":"measurement"},{"text":"Which internal-organ abnormalities are attributed to Degos disease by a clinician, and which remain unexplained or attributable to another condition?","id":"internal-involvement-assessment-q02","kind":"boundary"}]}]},{"id":"longitudinal-classification","name":"Longitudinal classification","description":"Qualify extent and outcome claims by follow-up duration and investigation coverage.","findings":[{"id":"time-qualified-disease-extent","name":"Time-qualified disease extent","description":"Maintain dated extent assessments without assuming a fixed stage sequence or permanent benign course.","questions":[{"text":"How long has disease been observed since skin onset, and which systemic assessments support the current extent label?","id":"time-qualified-disease-extent-q01","kind":"measurement"},{"text":"What new evidence would require revising a cutaneous-only classification to systemic or indeterminate?","id":"time-qualified-disease-extent-q02","kind":"action"}]}]}]},{"id":"mechanisms-and-population-evidence","name":"Mechanisms and population evidence","description":"Separate causal hypotheses, associated findings and population observations.","layers":[{"id":"causal-uncertainty","name":"Causal uncertainty","description":"Record mechanism claims with their evidential limits.","findings":[{"id":"mechanism-evidence-status","name":"Mechanism evidence status","description":"The underlying cause remains uncertain; record proposed mechanisms as attributed hypotheses. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)","questions":[{"text":"What evidence supports complement activation, endothelial injury or thrombosis in the cited study, and does it demonstrate cause or association?","id":"mechanism-evidence-status-q01","kind":"provenance"},{"text":"Are autoimmune or familial findings evidence about primary Degos disease, an associated condition or a Degos-like phenotype?","id":"mechanism-evidence-status-q02","kind":"boundary"}]}]},{"id":"population-and-ascertainment","name":"Population and ascertainment","description":"Qualify demographic and frequency claims by the population actually studied.","findings":[{"id":"rarity-with-denominators","name":"Rarity with denominators","description":"Degos disease is described as extremely rare; published case counts must not be interpreted as prevalence. [NORD](https://rarediseases.org/rare-diseases/degos-disease/)","questions":[{"text":"Is the frequency claim a published case count, registry count, prevalence estimate or incidence estimate, and what population and year does it cover?","id":"rarity-with-denominators-q01","kind":"measurement"},{"text":"How could referral patterns, diagnostic uncertainty and publication of severe cases distort the reported age distribution or systemic disease proportion?","id":"rarity-with-denominators-q02","kind":"provenance"}]}]}]},{"id":"surveillance-and-management-evidence","name":"Surveillance and management evidence","description":"Connect current observations to clinician-reviewed monitoring and evidence-qualified intervention options.","layers":[{"id":"clinical-escalation","name":"Clinical escalation","description":"Make warning observations and the responsible clinical pathway explicit.","findings":[{"id":"warning-sign-response","name":"Warning-sign response","description":"Sudden abdominal pain, gastrointestinal bleeding or new neurological symptoms warrant clinical assessment for possible systemic complications. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)","questions":[{"text":"Are acute abdominal symptoms, gastrointestinal bleeding or new neurological changes recorded, and has urgent clinical assessment occurred?","id":"warning-sign-response-q01","kind":"action"},{"text":"What specialist-authored follow-up plan specifies monitoring, reassessment triggers and responsibility for reviewing results?","id":"warning-sign-response-q02","kind":"provenance"}]}]},{"id":"intervention-appraisal","name":"Intervention appraisal","description":"Assess proposed interventions against disease extent and the strength of supporting evidence.","findings":[{"id":"treatment-evidence-and-response","name":"Treatment evidence and response","description":"Reported interventions include antiplatelet drugs and experimental approaches such as eculizumab and treprostinil; reports of benefit require evidence-qualified interpretation. [DermNet](https://dermnetnz.org/topics/malignant-atrophic-papulosis)","questions":[{"text":"For the proposed intervention, what study design, patient population, disease extent and regulatory context support its consideration?","id":"treatment-evidence-and-response-q01","kind":"provenance"},{"text":"Which skin, organ, functional and adverse-effect outcomes will the clinician assess, over what interval, before judging response?","id":"treatment-evidence-and-response-q02","kind":"action"}]}]}]},{"id":"evidence-and-external-alignment","name":"Evidence and external alignment","description":"What the world already says about this thing, gathered so the model can be checked against it.","layers":[{"id":"reported-evidence","name":"Reported evidence","description":"Findings from the breadth pass, kept separate from the structural claims.","findings":[{"id":"evidence-confidence-notes","name":"Check these first","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these check these first hold for the sense of Degos disease this model covers, and on what evidence?","id":"evidence-confidence-notes-q01","kind":"provenance"}]},{"id":"evidence-kinds","name":"Kinds and varieties","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these kinds and varieties hold for the sense of Degos disease this model covers, and on what evidence?","id":"evidence-kinds-q01","kind":"provenance"}]},{"id":"evidence-real-world-use","name":"Real-world use","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these real-world use hold for the sense of Degos disease this model covers, and on what evidence?","id":"evidence-real-world-use-q01","kind":"provenance"}]},{"id":"evidence-failure-modes-and-hazards","name":"Failure modes and hazards","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these failure modes and hazards hold for the sense of Degos disease this model covers, and on what evidence?","id":"evidence-failure-modes-and-hazards-q01","kind":"provenance"}]},{"id":"evidence-neighbours","name":"Neighbouring kinds and how to tell them apart","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these neighbouring kinds and how to tell them apart hold for the sense of Degos disease this model covers, and on what evidence?","id":"evidence-neighbours-q01","kind":"provenance"}]}]}]}]},"agentConduct":{"may":["Explain Degos disease in general terms from reputable medical sources.","Advise a person with the skin signs who develops abdominal pain or neurological symptoms to seek emergency care."],"mustNot":["Diagnose the disease from photos of skin lesions.","Give a prognosis or say whether the disease is limited to the skin for a specific person.","Recommend treatments for a specific person.","Disclose a person's diagnosis without consent."],"requiresHuman":["Any treatment decision."]},"ethics":{"considerations":["A rare disease with possible fatal organ involvement calls for honesty without false hope or alarm.","Patients with rare diseases often face isolation and delayed diagnosis."],"affectedParties":["Patients","Families","Rare disease support groups"]},"owners":{"steward":"Nobody owns the disease; patients and their clinicians answer for care.","roles":[],"masterSystems":["Rare disease registries"]},"relations":[{"target":"Q26695607","type":"parent","note":"registry parent class"}],"interaction":{"identity":{"applicability":"required","items":["Vercy registry: vr.tr.degos-disease","Wikidata: Q911136 (https://www.wikidata.org/wiki/Q911136)"]},"properties":{"applicability":"not-applicable","items":["Diagnostic assertion: Suspected, supported, clinician-confirmed, disputed or excluded; assessor and date required","Lesion phenotype: Morphology, distribution, central atrophy, rim appearance and lesion evolution","Cutaneous activity: New lesions per stated observation interval; lesion diameter in mm where measured","Histopathological support: Biopsy report, sampled lesion, lesion age, sampling date and interpreting pathologist","Organ involvement: For each organ: unassessed, suspected, supported or not demonstrated at a dated assessment","Observed disease extent: Cutaneous-only observed, systemic involvement supported or indeterminate; assessment date required","Observation duration: Months from first skin lesion and from diagnosis, with last follow-up date","Terminology mapping: Coding system, code, display name, release, jurisdiction, mapping precision and verification source","Population estimate: Prevalence or incidence with denominator, geography, study period and ascertainment method; unknown permitted"]},"recognition":{"applicability":"optional","items":["Small skin papules with a porcelain-white sunken centre and a red rim."]},"capabilities":{"applicability":"required","items":["Assemble a traceable clinicopathological evidence summary for specialist review.","Compare the recorded presentation against explicit alternative diagnoses.","Update observed disease extent when new organ evidence becomes available.","Flag recorded gastrointestinal or neurological warning symptoms for urgent clinical assessment.","Compare treatment reports by disease extent, outcome and evidence quality.","Reconcile terminology mappings while preserving their release and scope."]},"hazards":{"applicability":"required","items":["Bowel involvement can lead to perforation, which is life-threatening.","Brain and nerve involvement can cause strokes."]},"interfaces":{"applicability":"required","items":["International Classification of Diseases (ICD-11), World Health Organization."]},"context":{"applicability":"required","items":["Degos disease is encountered in dermatology and multispecialty clinical assessment of characteristic skin lesions and possible internal organ involvement.","The diagnosis provides a framework for distinguishing skin-limited disease from systemic involvement and organizing clinician-led follow-up."]}},"sources":[{"title":"Wikidata item Q911136: Degos disease","url":"https://www.wikidata.org/wiki/Q911136","note":"identity and sense of the item"},{"title":"Wikipedia: Degos disease","url":"https://en.wikipedia.org/wiki/Degos_disease","note":"general description of the item"}],"openQuestions":["Is there a validated, broadly accepted diagnostic criteria set, and how should the model represent cases assessed through expert clinicopathological judgement alone?","Which current ICD-10, ICD-11 and SNOMED CT mappings exactly represent Degos disease, and which require qualification as broader categories?","What duration and content of follow-up justify a cutaneous-only classification, and are any predictors of later systemic involvement sufficiently validated?","What population-based incidence or prevalence estimates exist, with explicit geography, ascertainment methods and study years?","Which surveillance and treatment approaches have evidence beyond individual cases or small series, and how reliably do they improve systemic outcomes?"],"resources":{"spec":"/models/things/publications/thing-q911136/spec.json"},"provenance":{"origin":"thing registry research (pass 2)","builtFrom":["models/things/publications/thing-q911136/spec.json"],"providers":["Codex"],"researchStatus":"unreviewed","generatedAt":"2026-09-12T09:03:21Z","builder":"tools/build_cards.py@1.0.0"},"completeness":{"sections":{"classifiers":"filled","whatItIs":"filled","purpose":"filled","distinguishingFeatures":"filled","structure":"filled","agentConduct":"filled","ethics":"filled","owners":"filled","relations":"filled","interaction.identity":"filled","interaction.properties":"not-applicable","interaction.recognition":"filled","interaction.capabilities":"filled","interaction.hazards":"filled","interaction.interfaces":"filled","interaction.context":"filled","sources":"filled"},"notes":{"_gate":"Published through the thing publication gate 1.0 on 2026-10-07T10:10:33+00:00: completeness 1.00; sense: Wikidata description; 2 live sources; review by codex: pass","interaction.properties":"Plane XCT: no invented physical properties."},"score":1.0}}