{"Name":"Linear scleroderma","DiseaseID__c":"GARD:0009513","id":9513,"encodedName":"linear-scleroderma","IsDeleted":false,"Disease_Name_Full__c":"Linear scleroderma","Xref_IDs__c":"22784002; C0263409; C116780; L94.1; MEDGEN:75518; MONDO:0043294","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":0,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0043294","Disease_Description__c":"A type of localized scleroderma characterized by a long strip of indurated skin, which is typically found unilaterally on an arm or leg, and sometimes on the forehead or trunk. This disorder often affects the tissues beneath the skin, causing damage to bones, muscle or other organs. It can limit movement, alter growth, and disfigure the affected area.","GARD_Name__c":"Linear scleroderma","GARD_Synonym__c":"linear morphea","Curated_Disease_Description_Source__c":"GARD:0009513","Curated_Disease_Description__c":"Linear scleroderma is one sub-type of localized scleroderma, most commonly occurring in childhood. It is characterized by abnormalities of the skin and subcutaneous tissues that often follow a dermatomal distribution and that are found on one side of the body. Besides the lesion in the face or scalp there are also abnormalities of the muscles, fat tissue and skull. When the face is affected, some strips located on the forehead may be hollow and lead to an appearance termed \"en coup de sabre\". In most cases, Raynaud's phenomenon is absent. The exact cause is unknown.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":null,"Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0043294","ORPHANET_ID__c":null,"Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":null,"Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":null,"Spanish_GARD_Synonym__c":null,"Category_Linearization__c":null,"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":"Linear scleroderma is one sub-type of localized scleroderma, most commonly occurring in childhood. It is characterized by abnormalities of the skin and subcutaneous tissues that often follow a dermatomal distribution and that are found on one side of the body. Besides the lesion in the face or scalp there are also abnormalities of the muscles, fat tissue and skull. When the face is affected, some strips located on the forehead may be hollow and lead to an appearance termed \"en coup de sabre\". In most cases, Raynaud's phenomenon is absent. The exact cause is unknown.","Curated_Disease_Description_Source__c":"GARD:0009513","GARD_Synonym__c":"linear morphea","Name":"Linear scleroderma","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"National Scleroderma Foundation","Website__c":"https://www.scleroderma.org"},{"Account_Name__c":"Scleroderma Research Foundation","Website__c":"https://srfcure.org/"},{"Account_Name__c":"Scleroderma Society of Ontario","Website__c":"https://www.hardword.ca/"},{"Account_Name__c":"Scleroderma & Raynaud's UK","Website__c":"https://www.sruk.co.uk"},{"Account_Name__c":"Scleroderma Canada","Website__c":"https://www.scleroderma.ca/"}],"External_Identifier_Disease__c":[{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=22784002","Source__c":"C0263409; MONDO:0043294","Xref__c":"22784002"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0263409","Source__c":"C0263409","Xref__c":"C0263409"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C116780","Source__c":"C0263409; MONDO:0043294","Xref__c":"C116780"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=75518","Source__c":"C0263409","Xref__c":"MEDGEN:75518"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0043294","Source__c":"GARD:0009513","Xref__c":"MONDO:0043294"},{"URL__c":"http://purl.bioontology.org/ontology/ICD10CM/L94.1","Source__c":"MONDO:0043294","Xref__c":"L94.1"}],"tags":{},"synonyms":["linear morphea"]}