{
  "id": 9078,
  "label": "hereditary progressive mucinous histiocytosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0007725",
  "properties": {
    "xrefs": [
      "GARD:0016989",
      "MEDGEN:326771",
      "MESH:C564186",
      "OMIM:142630",
      "Orphanet:158025",
      "UMLS:C1840586",
      "icd11.foundation:284196883"
    ],
    "synonyms": [
      "histiocytosis, progressive mucinous"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      }
    ],
    "definition": "Hereditary progressive mucinous histiocytosis is a rare, benign, non-Langerhans cell histiocytosis characterized by childhood or adolescence onset of multiple, small, asymptomatic, slowly progressing, skin-colored to red-brown papules with predilection for the face, dorsal hands, forearms and legs, without associated mucosal or visceral involvement. Histologically, papules are well-circumscribed, unencapsulated, nodular aggregates of histiocytes with abundant mucin in the upper and middermis."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 16345,
      "label": "non-Langerhans cell histiocytosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4688
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:4330",
          "GARD:0008231",
          "ICD9:288.4",
          "MEDGEN:9265",
          "MESH:D015616",
          "Orphanet:157987",
          "SCTID:127069007",
          "UMLS:C0019624"
        ],
        "synonyms": [
          "non-Langerhans-cell histiocytosis",
          "histiocytosis, non-Langerhans-cell"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Group of disorders which feature accumulations of active HISTIOCYTES and LYMPHOCYTES, but where the histiocytes are not LANGERHANS CELLS. The group includes HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; SINUS HISTIOCYTOSIS; xanthogranuloma; reticulohistiocytoma; juvenile XANTHOGRANULOMA; xanthoma disseminatum; as well as the lipid storage diseases (SEA-BLUE HISTIOCYTE SYNDROME; and NIEMANN-PICK DISEASES)."
      },
      "child_count": 15,
      "reference_id": "MONDO:0015531"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 16345,
      "label": "non-Langerhans cell histiocytosis"
    }
  ]
}