{
  "id": 11227,
  "label": "generalized peeling skin syndrome",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0010033",
  "properties": {
    "xrefs": [
      "GARD:0012862",
      "MEDGEN:930825",
      "Orphanet:263543",
      "SCTID:718749004",
      "UMLS:C4305156"
    ],
    "synonyms": [
      "generalised PSS",
      "generalised deciduous skin",
      "generalized PSS",
      "generalized deciduous skin",
      "peeling skin syndrome type 1",
      "PSS1",
      "peeling skin syndrome 1"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      }
    ],
    "definition": "Generalized peeling skin syndrome (PSS) is a form of PSS presenting with a generalized distribution. It comprises two sub-types: the non-inflammatory (PSS type A) and the inflammatory (PSS type B) form. PSS type A is characterized by generalized white scaling with superficial peeling of the skin, while PSS type B is characterized by superficial patchy peeling of the entire skin with underlying erythroderma, associated with pruritus, and atopy."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 19185,
      "label": "peeling skin syndrome",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16624
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060283",
          "GARD:0007347",
          "ICD9:757.39",
          "MEDGEN:336530",
          "NORD:1562",
          "OMIMPS:270300",
          "Orphanet:817",
          "SCTID:239065004",
          "UMLS:C1849193",
          "icd11.foundation:523640904"
        ],
        "synonyms": [
          "PSS",
          "deciduous skin",
          "familial continuous skin peeling syndrome",
          "idiopathic deciduous skin",
          "keratosis exfoliativa congenita",
          "peeling skin disease",
          "familial continuous skin peeling",
          "skin peeling syndrome"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Peeling skin syndrome (PSS) refers to a group of rare autosomal recessive forms of ichthyosis that is characterized clinically by superficial, asymptomatic, spontaneous peeling of the skin and histologically by a shedding of the outer layers of the epidermis. PSS presents with either an acral (acral PSS) or a generalized distribution (generalized PSS type A (non inflammatory) or B (inflammatory)). Some cases remain difficult to classify, suggesting that there could be additional subtypes of PSS."
      },
      "child_count": 5,
      "reference_id": "MONDO:0019347"
    }
  ],
  "children": [
    {
      "id": 15553,
      "label": "peeling skin syndrome type A",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        11227
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0070522",
          "GARD:0017258",
          "MEDGEN:864166",
          "OMIM:616265",
          "Orphanet:263548",
          "UMLS:C4015729"
        ],
        "synonyms": [
          "PSS type A",
          "generalised deciduous skin type A",
          "generalised peeling skin syndrome type A",
          "generalized deciduous skin type A",
          "generalized peeling skin syndrome type A",
          "non-inflammatory generalised peeling skin syndrome type A.",
          "non-inflammatory generalized peeling skin syndrome type A.",
          "non-inflammatory peeling skin syndrome type A",
          "peeling skin syndrome type 3",
          "PSS3",
          "peeling skin syndrome 3"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Peeling skin syndrome (PSS) type A is a non inflammatory form of generalized PSS, a type of ichthyosis, characterized by generalized white scaling and superficial painless peeling of the skin."
      },
      "child_count": 0,
      "reference_id": "MONDO:0014555"
    },
    {
      "id": 17404,
      "label": "generalized peeling skin syndrome type C",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        11227
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020904",
          "Orphanet:263558"
        ],
        "synonyms": [
          "generalised deciduous skin type C",
          "generalized deciduous skin type C",
          "peeling skin syndrome type C"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0016993"
    },
    {
      "id": 21493,
      "label": "peeling skin syndrome 1",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        11227
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0070520",
          "GARD:0017259",
          "MEDGEN:1885521",
          "OMIM:270300",
          "Orphanet:263553",
          "UMLS:C5679693"
        ],
        "synonyms": [
          "CDSN peeling skin syndrome",
          "PSS type B",
          "generalised deciduous skin type B",
          "generalised peeling skin syndrome type B",
          "generalized deciduous skin type B",
          "generalized peeling skin syndrome type B",
          "inflammatory peeling skin syndrome",
          "peeling skin syndrome 1",
          "peeling skin syndrome caused by mutation in CDSN",
          "peeling skin syndrome type B",
          "PSS",
          "PSS1",
          "deciduous skin",
          "keratolysis exfoliativa congenita",
          "skin peeling, familial continuous generalised",
          "skin peeling, familial continuous generalized"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Any peeling skin syndrome in which the cause of the disease is a mutation in the CDSN gene."
      },
      "child_count": 0,
      "reference_id": "MONDO:0024548"
    }
  ],
  "roots": [
    {
      "id": 19185,
      "label": "peeling skin syndrome"
    }
  ]
}