{
  "id": 18480,
  "label": "lichen myxedematosus",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018432",
  "properties": {
    "xrefs": [
      "GARD:0021709",
      "ICD9:701.8",
      "MEDGEN:1848864",
      "Orphanet:402007",
      "SCTID:111197009",
      "UMLS:C5848049"
    ],
    "synonyms": [
      "lichen myxoedematosus",
      "lichenoid myxedema",
      "papular mucinosis"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      }
    ]
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 4593,
      "label": "cutaneous mucinosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6820
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3141",
          "ICD9:701.8",
          "MEDGEN:56520",
          "MESH:D017520",
          "SCTID:402721001",
          "UMLS:C0162855",
          "icd11.foundation:2018255084"
        ],
        "synonyms": [
          "mucinosis",
          "mucinosis affecting the skin",
          "mucinoses"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "The mucinoses are a diverse group of disorders which have in common the deposition of basophilic, finely granular and stringy material (mucin) in the connective tissues of the dermis (dermal mucinoses), in the pilosebaceous follicles (follicular mucinoses), or in the epidermis and tumors derived therefrom (epithelial mucinoses)."
      },
      "child_count": 5,
      "reference_id": "MONDO:0002523"
    },
    {
      "id": 20387,
      "label": "dermis disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6820
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "MEDGEN:1843083",
          "Orphanet:79381",
          "UMLS:C5681483"
        ],
        "synonyms": [
          "dermis disease",
          "dermis disease or disorder",
          "disease of dermis",
          "disease or disorder of dermis",
          "disorder of dermis",
          "other dermis disorder"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "A disease that involves the dermis."
      },
      "child_count": 28,
      "reference_id": "MONDO:0021154"
    }
  ],
  "children": [
    {
      "id": 16442,
      "label": "scleromyxedema",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18480
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0007615",
          "ICD9:701.8",
          "MEDGEN:120476",
          "MESH:D053718",
          "MedDRA:10055046",
          "NCIT:C85061",
          "Orphanet:167635",
          "SCTID:402468007",
          "UMLS:C0263390"
        ],
        "synonyms": [
          "Arndt-Gottron disease",
          "Scleromyxedema",
          "generalised lichenoid papular eruption",
          "generalised papular and sclerodermoid lichen myxedematosus",
          "generalized lichenoid papular eruption",
          "generalized papular and sclerodermoid lichen myxedematosus",
          "scleromyxoedema",
          "generalised papular and sclerodermoid",
          "generalized papular and sclerodermoid",
          "lichen myxedematosus",
          "mucinosis, papular",
          "myxedematosus, lichen",
          "papular mucinosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Scleromyxedema is a rare, severe skin disorder. Signs and symptoms include abnormal accumulation of mucin in the skin (mucinosis), causing papular and sclerodermoid bumps; increased production of fibroblasts (connective tissue cells) in the absence of a thyroid disorder; and monoclonal gammopathy (abnormal proteins in the blood). It often involves internal organs and may affect various body systems. The cause of scleromyxedema is not known. There is no standard treatment. Management may involve the use of intravenous immunoglobulin (IVIG), plasmapheresis, thalidomide and corticoids, or more aggressive interventions, such as autologous bone marrow transplantation."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015665"
    },
    {
      "id": 19272,
      "label": "localized lichen myxedematosus",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18480
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0007321",
          "MEDGEN:1802421",
          "Orphanet:86795",
          "UMLS:C5575846",
          "icd11.foundation:15830032"
        ],
        "synonyms": [
          "papular mucinosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Localized lichen myxedematosus is a group of skin diseases characterized by the development of papules, nodules and/or plaques with mucin deposits and a variable degree of fibrosis in the absence of thyroid disease. The group comprises five sub-forms: nodular lichen myxedematosus, discrete papular lichen myxedematosus, papular mucinosis of infancy, acral persistent papular mucinosis and self-healing papular mucinosis."
      },
      "child_count": 5,
      "reference_id": "MONDO:0019446"
    },
    {
      "id": 19273,
      "label": "atypical lichen myxedematosus",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18480
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019066",
          "MEDGEN:1383234",
          "Orphanet:86797",
          "SCTID:725148000",
          "UMLS:C4510874"
        ],
        "synonyms": [
          "Intermediate lichen myxedematosus"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Atypical lichen myxedematosus is an intermediate form of lichen myxedematosus (LM) (a form of mucin dermal deposit) which does not meet the criteria for either scleromyxedema or the localized form. Three clinical subtypes have been described and include scleromyxedema without monoclonal gammopathy; localized forms with monoclonal gammopathy and/or systemic symptoms; localized forms with mixed features of the 5 subtypes of localized LM (discrete form, acral persistent papular mucinosis, self-healing papular mucinosis, papular mucinosis of infancy, and a pure nodular form). The course of atypical LM is unpredictable because only a few cases have been reported."
      },
      "child_count": 3,
      "reference_id": "MONDO:0019447"
    }
  ],
  "roots": [
    {
      "id": 4593,
      "label": "cutaneous mucinosis"
    },
    {
      "id": 20387,
      "label": "dermis disorder"
    }
  ]
}