{"Name":"Generalized peeling skin syndrome","DiseaseID__c":"GARD:0012862","id":12862,"encodedName":"generalized-peeling-skin-syndrome","IsDeleted":false,"Disease_Name_Full__c":"Generalized peeling skin syndrome","Xref_IDs__c":"718749004; C4305156; MEDGEN:930825; MONDO:0010033; ORPHA:263543","USA_Estimate__c":"1,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"1 to 8,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":7,"No_of_Age_at_Onset__c":2,"Description_Source__c":"MONDO:0010033","Disease_Description__c":"A form of peeling skin syndrome characterized by 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 non-inflammatory peeling of the skin, while PSS type B is characterized by superficial patchy peeling of the entire skin with underlying erythroderma, pruritus, and atopy.","GARD_Name__c":"Generalized peeling skin syndrome","GARD_Synonym__c":"generalised deciduous skin; generalised pss; generalized deciduous skin; generalized pss; peeling skin syndrome type 1","Curated_Disease_Description_Source__c":"MONDO:0010033","Curated_Disease_Description__c":"A form of peeling skin syndrome characterized by 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 non-inflammatory peeling of the skin, while PSS type B is characterized by superficial patchy peeling of the entire skin with underlying erythroderma, pruritus, and atopy.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"1,000","Age_at_Onset_Snippet_Text__c":"as a Newborn and as an Infant","SourceID__c":"ORPHA:263543","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0010033","ORPHANET_ID__c":"ORPHA:263543","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Síndrome de descamación cutánea generalizada","Spanish_Description_Source__c":"ORPHA:263543","Spanish_Description__c":"Es una forma de síndrome de descamación de la piel caracterizado por una distribución generalizada. Comprende dos subtipos: la forma no inflamatoria (PSS tipo A) y la inflamatoria (PSS tipo B). El PSS tipo A se caracteriza por descamación blanca generalizada con descamación superficial no inflamatoria de la piel, mientras que el PSS tipo B se caracteriza por descamación superficial en parches de toda la piel con eritrodermia subyacente, prurito y atopia.","Spanish_Disease_Name__c":"síndrome de descamación cutánea generalizada","Spanish_GARD_Synonym__c":"piel decidual generalizada; pss generalizado","Category_Linearization__c":"ORPHA:89826","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"A form of peeling skin syndrome characterized by 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 non-inflammatory peeling of the skin, while PSS type B is characterized by superficial patchy peeling of the entire skin with underlying erythroderma, pruritus, and atopy.","Curated_Disease_Description_Source__c":"MONDO:0010033","GARD_Synonym__c":"generalised deciduous skin; generalised pss; generalized deciduous skin; generalized pss; peeling skin syndrome type 1","Name":"Generalized peeling skin syndrome","Curated_USA_Estimate__c":"1,000","estimateUsa":"1,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"American Skin Association","Website__c":"https://www.americanskin.org/"},{"Account_Name__c":"Foundation for Ichthyosis and Related Skin Types","Website__c":"https://www.firstskinfoundation.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Genetics","Tag_Category__c":"Cause;Disease Category;Specialist","category_description":"Genetic diseases affect the DNA, or genetic instructions, which directs how tissues, organs, and body systems function.","curated_tag_name":"Genetic diseases"},{"Tag_Name__c":"Dermatology","Tag_Category__c":"Account;Disease Category;Specialist","category_description":"Skin diseases, or integumentary system diseases, affect the skin, hair, nails, sweat glands, or oil glands.","curated_tag_name":"Skin diseases"},{"Tag_Name__c":"Ichthyosis","Tag_Category__c":"Account","curated_tag_name":"Ichthyosis"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Neonatal","Provided_By__c":"ORPHA:263543"},{"Age_At_Onset__c":"Infancy","Provided_By__c":"ORPHA:263543"}],"External_Identifier_Disease__c":[{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C4305156","Source__c":"C4305156","Xref__c":"C4305156"},{"URL__c":"https://www.orpha.net/en/disease/detail/263543","Source__c":"C4305156; MONDO:0010033; ORPHA:263543","Xref__c":"ORPHA:263543"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=718749004","Source__c":"C4305156; MONDO:0010033","Xref__c":"718749004"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=930825","Source__c":"C4305156","Xref__c":"MEDGEN:930825"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0010033","Source__c":"GARD:0012862","Xref__c":"MONDO:0010033"}],"Inheritance__c":["Autosomal recessive"],"tags":{"Cause":["Genetics"],"Disease Category":["Genetics","Dermatology"],"Specialist":["Genetics","Dermatology","Pediatrics"],"Account":["Dermatology","Ichthyosis"]},"synonyms":["generalised deciduous skin"," generalised pss"," generalized deciduous skin"," generalized pss"," peeling skin syndrome type 1"]}