{"Name":"Dermatitis herpetiformis, familial","DiseaseID__c":"GARD:0001917","id":1917,"encodedName":"dermatitis-herpetiformis-familial","IsDeleted":false,"Disease_Name_Full__c":"Dermatitis herpetiformis, familial","Xref_IDs__c":"C1832586; C538218; MEDGEN:371361; MONDO:0011024; OMIM:601230","USA_Estimate__c":"200,000","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":"800,000 to 5,000,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0011024","Disease_Description__c":"Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. These are more common on the knees, elbows, buttocks and shoulder blades. The slow onset of symptoms usually begins during adulthood, but children can also be affected. Other symptoms mayinclude fluid-filled sores; red lesions that resemble hives; and itchiness, rednessand burning. The exact cause of this disease is not known,but it is frequently associated with the inability to digest gluten. People with this disease are typically treated with the drug dapsone.","GARD_Name__c":"Dermatitis herpetiformis, familial","GARD_Synonym__c":"dh; hereditary dermatitis herpetiformis","Curated_Disease_Description_Source__c":"GARD:0001917","Curated_Disease_Description__c":"Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. These are more common on the knees, elbows, buttocks and shoulder blades. Other symptoms may include fluid-filled sores; red lesions that resemble hives; and itchiness, redness and burning. The exact cause of this disease is not known, but it is frequently associated with the inability to digest gluten.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"200,000","Age_at_Onset_Snippet_Text__c":null,"SourceID__c":"ORPHA:1656","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0011024","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":"Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. These are more common on the knees, elbows, buttocks and shoulder blades. Other symptoms may include fluid-filled sores; red lesions that resemble hives; and itchiness, redness and burning. The exact cause of this disease is not known, but it is frequently associated with the inability to digest gluten.","Curated_Disease_Description_Source__c":"GARD:0001917","GARD_Synonym__c":"dh; hereditary dermatitis herpetiformis","Name":"Dermatitis herpetiformis, familial","Curated_USA_Estimate__c":"200,000","estimateUsa":"200,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Autoimmune Registry","Website__c":"https://www.autoimmuneregistry.org/"},{"Account_Name__c":"Global Autoimmune Institute","Website__c":"https://www.autoimmuneinstitute.org/"}],"External_Identifier_Disease__c":[{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1832586","Source__c":"C1832586","Xref__c":"C1832586"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=371361","Source__c":"C1832586","Xref__c":"MEDGEN:371361"},{"URL__c":"https://www.omim.org/entry/601230","Source__c":"C1832586; MONDO:0011024","Xref__c":"OMIM:601230"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C538218","Source__c":"MONDO:0011024","Xref__c":"C538218"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0011024","Source__c":"GARD:0001917","Xref__c":"MONDO:0011024"}],"Inheritance__c":["Autosomal dominant"],"GARD_Disease_Feature__c":[{"Provided_By__c":"OMIM:601230","Feature__r":{"HPO_Description__c":"Pruritus is an itch or a sensation that makes a person want to scratch. This term refers to an abnormally increased disposition to experience pruritus.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0000989","HPO_Synonym__c":"Itching; Itchy skin; Skin itching","HPO_Name__c":"Pruritus","Feature_System__c":"Skin System","HPO_Feature_Type__c":"Symptom"}}],"tags":{},"synonyms":["dh"," hereditary dermatitis herpetiformis"]}