{
  "id": 18765,
  "label": "pyoderma gangrenosum",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018824",
  "properties": {
    "xrefs": [
      "DOID:8553",
      "GARD:0007510",
      "ICD10CM:L88",
      "ICD10WHO:L88",
      "ICD9:686.01",
      "MEDGEN:43224",
      "MESH:D017511",
      "MedDRA:10037635",
      "NANDO:2200437",
      "NORD:1638",
      "Orphanet:48104",
      "SCTID:74578003",
      "UMLS:C0085652",
      "icd11.foundation:2120746218"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      },
      {
        "ref": "MONDO:0002254",
        "name": "syndromic disease"
      },
      {
        "ref": "MONDO:0003900",
        "name": "connective tissue disorder"
      }
    ],
    "definition": "Pyoderma gangrenosum (PG) is a primarily sterile inflammatory neutrophilic dermatosis characterized by recurrent cutaneous ulcerations with a mucopurulent or hemorrhagic exudate."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 4,
  "parents": [
    {
      "id": 4929,
      "label": "pyoderma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6820
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:4223",
          "ICD10CM:L08.0",
          "ICD9:686.0",
          "ICD9:686.00",
          "ICD9:686.09",
          "MEDGEN:18783",
          "MESH:D011711",
          "SCTID:70759006",
          "UMLS:C0034212",
          "icd11.foundation:1991248382"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Any skin disease that is pyegenic."
      },
      "child_count": 5,
      "reference_id": "MONDO:0002922"
    },
    {
      "id": 19503,
      "label": "autoinflammatory syndrome",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4370,
        7203
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0051000",
          "ICD10CM:M04-M04",
          "MEDGEN:855741",
          "MedDRA:10072220",
          "NANDO:2100156",
          "NCIT:C119050",
          "Orphanet:93665",
          "UMLS:C3890737"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0003900",
            "name": "connective tissue disorder"
          }
        ],
        "definition": "A group of disorders of the innate immune system characterized by attacks of seemingly unprovoked inflammation without significant levels of either autoantibodies or autoreactive T cells more characteristic of autoimmune disease."
      },
      "child_count": 74,
      "reference_id": "MONDO:0019751"
    }
  ],
  "children": [
    {
      "id": 22812,
      "label": "classic pyoderma gangrenosum",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18765
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0022220",
          "MEDGEN:1814456",
          "Orphanet:538863",
          "UMLS:C5680157"
        ],
        "synonyms": [
          "Ulcerative pyoderma gangrenosum"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0003900",
            "name": "connective tissue disorder"
          }
        ],
        "definition": "A rare subtype of pyoderma gangrenosum disease characterized by rapidly progressive, single or multiple, painful, aseptic ulcers which present overhanging, violaceous and undermined borders, surrounding induration and erythema, and granulation tissue (occasionally necrotic tissue and/or a purulent exudate) at the base, mainly affecting the legs (but other body surfaces may also be involved), leading to chronic ulcerations and often regressing with cribriform mutilating scars. The disease presents a chronic relapsing course and systemic features (e.g. fever, malaise, arthralgia, myalgia) may be associated."
      },
      "child_count": 0,
      "reference_id": "MONDO:0035235"
    },
    {
      "id": 22813,
      "label": "pustular pyoderma gangrenosum",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18765
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0022221",
          "MEDGEN:1814476",
          "Orphanet:538866",
          "UMLS:C5681317"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0003900",
            "name": "connective tissue disorder"
          }
        ],
        "definition": "A rare subtype of pyoderma gangrenosum characterized by multiple painful, sterile pustules with a surrounding erythematous halo, predominantly occurring on the trunk and extensor surfaces of the limbs, and potentially persisting for months. Histopathology shows a dermal neutrophilic infiltrate and subcorneal neutrophilic micropustules. The condition is commonly associated with inflammatory bowel disease."
      },
      "child_count": 0,
      "reference_id": "MONDO:0035236"
    },
    {
      "id": 22814,
      "label": "bullous pyoderma gangrenosum",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18765
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0022222",
          "MEDGEN:590609",
          "Orphanet:538869",
          "UMLS:C0406687"
        ],
        "synonyms": [
          "Phemphigoid pyoderma gangrenosum"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0003900",
            "name": "connective tissue disorder"
          }
        ],
        "definition": "A rare subtype of pyoderma gangrenosum disease characterized by grouped vesicles that rapidly spread and coalesce to form large bullae, which evolve into ulcerations that have an erythematous peripheral halo and central necrosis, mainly affecting the upper limbs and face. Lymphoproliferative diseases are frequently associated, thus prognosis is often compromised."
      },
      "child_count": 0,
      "reference_id": "MONDO:0035237"
    },
    {
      "id": 22815,
      "label": "vegetative pyoderma gangrenosum",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18765
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0022223",
          "MEDGEN:1842944",
          "Orphanet:538872",
          "UMLS:C5680158"
        ],
        "synonyms": [
          "Granulomatous pyoderma gangrenosum"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0003900",
            "name": "connective tissue disorder"
          }
        ],
        "definition": "A rare subtype of pyoderma gangrenosum disease characterized by a solitary, erythematous, ulcerated plaque, which lacks the violaceous border typically present in classic pyoderma gangrenosum, usually affecting individuals who are otherwise healthy. Histologically, the lesion presents a central layer containing neutrophilic inflammation, surrounded by a palisade of histiocytes, which are rimmed by a lymphocytic infiltrate. In comparison with the other variants of pyoderma gangrenosum, this subtype usually shows a good response to less aggressive treatments and underlying systemic disorders are less frequently associated. It is considered the most benign and uncommon clinical variant of pyoderma gangrenosum."
      },
      "child_count": 0,
      "reference_id": "MONDO:0035238"
    }
  ],
  "roots": [
    {
      "id": 4929,
      "label": "pyoderma"
    },
    {
      "id": 19503,
      "label": "autoinflammatory syndrome"
    }
  ]
}