{
  "id": 19156,
  "label": "diffuse cutaneous mastocytosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019315",
  "properties": {
    "xrefs": [
      "DOID:3665",
      "GARD:0012686",
      "ICDO:9740/1",
      "MEDGEN:44303",
      "MedDRA:10012812",
      "NCIT:C3218",
      "Orphanet:79456",
      "UMLS:C0024901",
      "icd11.foundation:193128939"
    ],
    "synonyms": [
      "DCM",
      "diffuse cutaneous maculopapulous mastocytosis",
      "diffuse cutaneous mastocytosis"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      },
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "Diffuse cutaneous mastocytosis (DCM) is a rare form of cutaneous mastocytosis (CM) characterized by generalized erythroderma, various degrees of blistering, skin with a ''peau d'orange'' appearance and the accumulation of mast cells in the skin. At least two DCM variants are recognized, one with extreme blistering (Bullous DCM) and one with infiltrations (Pseudoxanthomatous DCM)."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 18936,
      "label": "cutaneous mastocytosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4409,
        7148,
        9280
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3663",
          "EFO:1000886",
          "GARD:0007842",
          "HP:0200151",
          "ICD10CM:D47.01",
          "ICDO:9740/1",
          "MEDGEN:210143",
          "MESH:D034701",
          "NCIT:C7137",
          "OMIM:154800",
          "ONCOTREE:CMCD",
          "Orphanet:66646",
          "SCTID:397012002",
          "UMLS:C1136033",
          "icd11.foundation:1300710062"
        ],
        "synonyms": [
          "CM",
          "cutaneous (skin) mastocytosis",
          "cutaneous mastocytosis",
          "cutaneous mastocytosis (disease)",
          "mastocytosis, cutaneous",
          "mastocytosis, systemic, somatic",
          "CMCD"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Cutaneous mastocytosis is a term referring to a group of diseases characterized by abnormal accumulation and proliferation of skin mastocytes. In some cases (most commonly in adults), cutaneous mastocytosis may occur in association with mast cell infiltration of various extracutaneous organs, in which case the disorder is referred to as systemic mastocytosis."
      },
      "child_count": 9,
      "reference_id": "MONDO:0019023"
    }
  ],
  "children": [
    {
      "id": 17579,
      "label": "bullous diffuse cutaneous mastocytosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        19156
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021088",
          "MEDGEN:1781582",
          "Orphanet:280785",
          "UMLS:C5546764",
          "icd11.foundation:227748867"
        ],
        "synonyms": [
          "bullous DCM"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Bullous diffuse cutaneous mastocytosis (BDCM) is a form of diffuse cutaneous mastocytosis (DCM) characterized by generalized erythroderma and severe blistering associated with the accumulation of mast cells in the skin."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017243"
    },
    {
      "id": 17580,
      "label": "pseudoxanthomatous diffuse cutaneous mastocytosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        19156
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021089",
          "MEDGEN:1842453",
          "Orphanet:280794",
          "UMLS:C5679949"
        ],
        "synonyms": [
          "Pseudoxanthomatous DCM",
          "infiltrative small vesicular DCM",
          "infiltrative small vesicular diffuse cutaneous mastocytosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Pseudoxanthomatous diffuse cutaneous mastocytosis (PDCM) is a rare form of diffuse cutaneous mastocytosis (DCM) characterized by yellow-orange infiltrated and xanthogranuloma-like lesions with only limited blistering."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017244"
    }
  ],
  "roots": [
    {
      "id": 18936,
      "label": "cutaneous mastocytosis"
    }
  ]
}