{"Name":"Necrobiotic xanthogranuloma","DiseaseID__c":"GARD:0010951","id":10951,"encodedName":"necrobiotic-xanthogranuloma","IsDeleted":false,"Disease_Name_Full__c":"Necrobiotic xanthogranuloma","Xref_IDs__c":"404164003; C1275339; D058252; MEDGEN:266158; MONDO:0015537; ORPHA:158011","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":2,"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":3,"Disease_Characteristics_Score__c":3,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0015537","Disease_Description__c":"A cutaneous necrobiotic disorder characterized by firm, yellow plaques or nodules, often in a periorbital distribution. It is often accompanied by an elevated erythrocyte sedimentation rate; leukopenia; and monoclonal gammopathy (IgG-kappa type) and systemic involvement.","GARD_Name__c":"Necrobiotic xanthogranuloma","GARD_Synonym__c":null,"Curated_Disease_Description_Source__c":"GARD:0010951","Curated_Disease_Description__c":"Necrobiotic xanthogranuloma (NXG) is a rare, chronic form of non-Langerhans histiocytosis usually found in older adults. Xanthogranulomas are lesions made of immune cells known as a histiocytes. The term necrobiotic refers to the buildup of broken down collagen fibers that can be seen under a microscope. The typical lesion is a yellow, thickened, or raised lesion (plaque) located around the eyes. In most cases the lesions are associated with conditions in which abnormal proteins are found in the blood (monoclonal gammopathies). NXG is also sometimes associated with blood cancers or lymphoproliferative disorders. Approximately 50% of people with NXG have problems with their eyes, such as burning or itching, blurred vision, double vision, bulging of the eyeball, drooping of the eyelid, and restricted eye movement. In most cases, other parts of the body, such as the trunk, legs, face, and arms, are also involved. Internal organs may sometimes be affected.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":"ORPHA:158011","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0015537","ORPHANET_ID__c":"ORPHA:158011","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Xantogranuloma necrobiótico","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"xantogranuloma necrobiótico","Spanish_GARD_Synonym__c":null,"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":"Necrobiotic xanthogranuloma (NXG) is a rare, chronic form of non-Langerhans histiocytosis usually found in older adults. Xanthogranulomas are lesions made of immune cells known as a histiocytes. The term necrobiotic refers to the buildup of broken down collagen fibers that can be seen under a microscope. The typical lesion is a yellow, thickened, or raised lesion (plaque) located around the eyes. In most cases the lesions are associated with conditions in which abnormal proteins are found in the blood (monoclonal gammopathies). NXG is also sometimes associated with blood cancers or lymphoproliferative disorders. Approximately 50% of people with NXG have problems with their eyes, such as burning or itching, blurred vision, double vision, bulging of the eyeball, drooping of the eyelid, and restricted eye movement. In most cases, other parts of the body, such as the trunk, legs, face, and arms, are also involved. Internal organs may sometimes be affected.","Curated_Disease_Description_Source__c":"GARD:0010951","Name":"Necrobiotic xanthogranuloma","estimateUsa":""}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Rheumatology","Tag_Category__c":"Specialist"},{"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"}],"External_Identifier_Disease__c":[{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1275339","Source__c":"C1275339","Xref__c":"C1275339"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C058252","Source__c":"C1275339; MONDO:0015537","Xref__c":"D058252"},{"URL__c":"https://www.orpha.net/en/disease/detail/158011","Source__c":"C1275339; MONDO:0015537; ORPHA:158011","Xref__c":"ORPHA:158011"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=404164003","Source__c":"C1275339; MONDO:0015537","Xref__c":"404164003"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=266158","Source__c":"C1275339","Xref__c":"MEDGEN:266158"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0015537","Source__c":"GARD:0010951","Xref__c":"MONDO:0015537"}],"tags":{"Specialist":["Rheumatology","Dermatology"],"Account":["Dermatology"],"Disease Category":["Dermatology"]},"synonyms":[""]}