{
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    "__licence": {
      "identifier": "CC-BY-4.0",
      "link": "https://creativecommons.org/licenses/by/4.0",
      "name": "Creative Commons Attribution 4.0 International"
    },
    "results": {
      "Date": "2026-06-23 07:53:50",
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      "DisorderFlag": [
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          "DisorderMappingICDRefUrl": null,
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          "Reference": "I72.8",
          "Source": "ICD-10"
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          "DisorderMappingICDRefUrl": "https://icd.who.int/browse/latest-release/mms/en#1674517934%2funspecified",
          "DisorderMappingICDRelation": "Index term (ICD-10: Orphanet entity listed in the ICD-10 Index. ICD-11: Orphanet entity listed in the ICD-11 Foundation)",
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          "Reference": "BD50.Z",
          "Source": "ICD-11"
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          "DisorderMappingICDRefUrl": null,
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          "DisorderMappingRelation": "E (Exact mapping: the two concepts are equivalent)",
          "DisorderMappingValidationStatus": "Validated",
          "Reference": "0010889",
          "Source": "MONDO"
        },
        {
          "DisorderMappingICDRefUri": null,
          "DisorderMappingICDRefUrl": null,
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          "DisorderMappingRelation": "E (Exact mapping: the two concepts are equivalent)",
          "DisorderMappingValidationStatus": "Validated",
          "Reference": "600459",
          "Source": "OMIM"
        },
        {
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          "DisorderMappingRelation": "E (Exact mapping: the two concepts are equivalent)",
          "DisorderMappingValidationStatus": "Validated",
          "Reference": "C4304024",
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      ],
      "ORPHAcode": 1682,
      "OrphanetURL": "http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=1682",
      "Preferred term": "Arterial dissection-lentiginosis syndrome",
      "SummaryInformation": [
        {
          "Definition": "A rare association syndrome, reported in several members of two families to date, characterized by arterial dissection, occurring at an early age and presenting with a range of manifestations depending on the vascular territory involved (ex. headache, dysphasia, hemiparesis), in association with cystic medial necrosis and multiple lentigines (brown and black in color and mainly affecting the skin of the trunk and extremities)."
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      ],
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      "Typology": "Malformation syndrome"
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    "lang": "en",
    "name": "Rare diseases and cross-referencing"
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    "path": {
      "ORPHAcode": 1682
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    "query": {}
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  "uri": "https://api.orphadata.com/rd-cross-referencing/orphacodes/1682"
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