{
  "id": 8070,
  "label": "pemphigus",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0006594",
  "properties": {
    "xrefs": [
      "DOID:9182",
      "EFO:1000749",
      "GARD:0007352",
      "ICD10CM:L10",
      "ICD10WHO:L10",
      "ICD9:694.4",
      "MEDGEN:45369",
      "MESH:D010392",
      "NANDO:1200228",
      "NCIT:C34909",
      "SCTID:65172003",
      "UMLS:C0030807",
      "Wikipedia:Pemphigus",
      "icd11.foundation:191659986"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      },
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      }
    ],
    "definition": "Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals).This conditioncan occur at any age, but often strikes people in middle or older age. Studies have shown that some populations may be at greater risk for certain types of pemphigus. For instance, people of Jewish descent and those from India, Southeast Europe, and the Middle East are at greater risk for pemphigus vulargis, while pemphigus foliaceus is more common in North America, Turkey, and South America. Pemphigus is a chronic disease which is best controlled by early diagnosis and treatment.Treatment includes steroids to reduce inflammation,drugs that suppress the immune system responseand antibiotics to treat associated infections. There are four main types of pemphigus: Pemphigus vulgaris Pemphigus foliaceus IgA pemphigus Paraneoplastic pemphigus"
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 6,
  "parents": [
    {
      "id": 19177,
      "label": "autoimmune bullous skin disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4496,
        8092,
        8586
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:8502",
          "EFO:0008598",
          "GARD:0019028",
          "ICD9:694.8",
          "ICD9:694.9",
          "MEDGEN:1842418",
          "Orphanet:79669",
          "SCTID:7231009",
          "UMLS:C5681494"
        ],
        "synonyms": [
          "bullous skin disease",
          "bullous dermatosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "An autoimmune disease characterized by blisters on the skin."
      },
      "child_count": 33,
      "reference_id": "MONDO:0019337"
    }
  ],
  "children": [
    {
      "id": 8041,
      "label": "pemphigoid gestationis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        8070,
        21520
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0040098",
          "DOID:14482",
          "EFO:1000709",
          "GARD:0006497",
          "ICD10CM:O26.4",
          "ICD9:646.80",
          "MEDGEN:9233",
          "MESH:D006559",
          "MedDRA:10019939",
          "NCIT:C85003",
          "Orphanet:63275",
          "SCTID:86081009",
          "UMLS:C0019343",
          "Wikipedia:Gestational_pemphigoid",
          "icd11.foundation:1405755890"
        ],
        "synonyms": [
          "Herpes gestationis",
          "Herpes gestationis (disorder)",
          "Herpes gestationis NOS (disorder)",
          "Herpes gestationis unspecified (disorder)",
          "gestational pemphigoid",
          "pemphigoid gestationis",
          "pemphigus gestationis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "A rare pregnancy-associated autoimmune skin disease that is characterized by an itchy rash that develops into blisters. It is most common during the second and third trimesters of pregnancy. It was previously known as herpes gestationis although it has no association with the herpes virus whatsoever."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006558"
    },
    {
      "id": 9526,
      "label": "Hailey-Hailey disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        8070,
        19129,
        23867
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050429",
          "GARD:0006559",
          "MEDGEN:43100",
          "MESH:D016506",
          "NANDO:1200631",
          "NCIT:C82865",
          "NORD:1211",
          "OMIM:169600",
          "Orphanet:2841",
          "SCTID:79468000",
          "UMLS:C0085106",
          "icd11.foundation:818400628"
        ],
        "synonyms": [
          "Hailey-Hailey disease",
          "benign chronic familial pemphigus of Hailey-Hailey",
          "benign chronic pemphigus",
          "pemphigus, benign familial",
          "BCPM",
          "benign familial pemphigus",
          "familial benign chronic pemphigus",
          "familial benign pemphigus"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Benign chronic familial pemphigus of Hailey-Hailey is characterized by rhagades mostly located in the armpits, inguinal and perineal folds (scrotum, vulva)."
      },
      "child_count": 0,
      "reference_id": "MONDO:0008218"
    },
    {
      "id": 9527,
      "label": "pemphigus vulgaris",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        8070
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060851",
          "EFO:0004719",
          "GARD:0007355",
          "ICD10CM:L10.0",
          "MEDGEN:10621",
          "MESH:C536645",
          "MedDRA:10052802",
          "NANDO:1200229",
          "NCIT:C34910",
          "OMIM:169610",
          "Orphanet:704",
          "SCTID:49420001",
          "UMLS:C0030809",
          "icd11.foundation:278358681"
        ],
        "synonyms": [
          "pemphigus vulgaris, familial"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Pemphigus is a group of chronic autoimmune skin diseases characterized by blister formations on the outer layer of the skin and the mucous membranes. Three clinical forms have been characterized, of which pemphigus vulgaris is the most frequent (75%)."
      },
      "child_count": 1,
      "reference_id": "MONDO:0008219"
    },
    {
      "id": 16763,
      "label": "herpetiform pemphigus",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        8070
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020406",
          "MEDGEN:1655315",
          "Orphanet:208524",
          "UMLS:C4749730"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Herpetiform pemphigus is a rare superficial pemphigus disease characterized by severe intractable pruritus with erythematous or urticarial plaques and vesicles organized in a herpetiform pattern. Mucosae are generally spared. Eosinophilia in peripheral blood and low titers of circulating autoantibodies are observed in many cases. Histologically, minimal or no apparent acantholysis is associated."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016164"
    },
    {
      "id": 19164,
      "label": "pemphigus erythematosus",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        8070
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0008603",
          "GARD:0019022",
          "ICD10CM:L10.4",
          "MEDGEN:537812",
          "MedDRA:10058917",
          "NANDO:1200233",
          "Orphanet:79480",
          "SCTID:36739006",
          "UMLS:C0263312",
          "icd11.foundation:399813106"
        ],
        "synonyms": [
          "Senear-Usher syndrome",
          "seborrheic pemphigus"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Pemphigus erythematosus is a rare superficial pemphigus disease characterized clinically by well-demarcated, localized, erythematous, scaly, hyperkeratotic, crusted plaques, with frequent butterfly distribution over the malar area of the face (but also commonly involving trunk and scalp, and less frequently the extremities, with a photoexposed distribution). Histologically, granular deposits along the dermal-epidermal junction, in addition to intercellular deposition in the upper epidermis, are observed."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019323"
    },
    {
      "id": 19165,
      "label": "pemphigus foliaceus",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        8070
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080850",
          "EFO:0008601",
          "GARD:0007354",
          "HGNC:3050",
          "MEDGEN:75513",
          "MedDRA:10057069",
          "NANDO:1200230",
          "Orphanet:79481",
          "SCTID:35154004",
          "UMLS:C0263313",
          "icd11.foundation:24246260"
        ],
        "synonyms": [
          "PF"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          }
        ],
        "definition": "Pemphigus foliaceous is a rare superficial pemphigus disease characterized by multiple, pruritic, scaly, crusted cutaneous erosions, with flaky circumscribed patches, localized mostly on the face, scalp, trunk and extremities, often presenting an erythematous base. Mucosal involvement is rarely observed."
      },
      "child_count": 1,
      "reference_id": "MONDO:0019324"
    }
  ],
  "roots": [
    {
      "id": 19177,
      "label": "autoimmune bullous skin disease"
    }
  ]
}