{"Name":"Erosive pustular dermatosis of the scalp","DiseaseID__c":"GARD:0002188","id":2188,"encodedName":"erosive-pustular-dermatosis-of-the-scalp","IsDeleted":false,"Disease_Name_Full__c":"Erosive pustular dermatosis of the scalp","Xref_IDs__c":"238733003; C0406464; MEDGEN:96057; MONDO:0016379; ORPHA:222","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":1,"Disease_Characteristics_Score__c":6,"No_of_Age_at_Onset__c":4,"Description_Source__c":"MONDO:0016379","Disease_Description__c":"Erosive pustular dermatosis of the scalp is a rare chronic inflammation of the scalp usually occurring in elderly women (>70 years old) and characterized by the development of painful pustules, shallow erosions, and crusting on atrophic skin that eventually result in cicatricial alopecia.","GARD_Name__c":"Erosive pustular dermatosis of the scalp","GARD_Synonym__c":null,"Curated_Disease_Description_Source__c":"MONDO:0016379","Curated_Disease_Description__c":"Erosive pustular dermatosis of the scalp is a rare chronic inflammation of the scalp usually occurring in elderly women (>70 years old) and characterized by the development of painful pustules, shallow erosions, and crusting on atrophic skin that eventually result in cicatricial alopecia.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":"at a variety of ages","SourceID__c":"ORPHA:222","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0016379","ORPHANET_ID__c":"ORPHA:222","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Dermatosis pustulosa erosiva de cuero cabelludo","Spanish_Description_Source__c":"ORPHA:222","Spanish_Description__c":"La dermatosis pustulosa erosiva de cuero cabelludo es una inflamación crónica poco frecuente del cuero cabelludo que normalmente ocurre en mujeres mayores (>70 años de edad) y que se caracteriza por el desarrollo de pústulas dolorosas, erosiones superficiales, y formación de costras en la piel atrófica que finalmente dan lugar a una alopecia cicatricial.","Spanish_Disease_Name__c":"dermatosis pustulosa erosiva de cuero cabelludo","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":"Erosive pustular dermatosis of the scalp is a rare chronic inflammation of the scalp usually occurring in elderly women (>70 years old) and characterized by the development of painful pustules, shallow erosions, and crusting on atrophic skin that eventually result in cicatricial alopecia.","Curated_Disease_Description_Source__c":"MONDO:0016379","Name":"Erosive pustular dermatosis of the scalp","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Scarring Alopecia Foundation","Website__c":"https://scarringalopecia.org/"}],"GARD_Disease_Tag__c":[{"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":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:222"},{"Age_At_Onset__c":"Adolescent","Provided_By__c":"ORPHA:222"},{"Age_At_Onset__c":"Childhood","Provided_By__c":"ORPHA:222"},{"Age_At_Onset__c":"Elderly","Provided_By__c":"ORPHA:222"}],"External_Identifier_Disease__c":[{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=238733003","Source__c":"C0406464; MONDO:0016379","Xref__c":"238733003"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0406464","Source__c":"C0406464","Xref__c":"C0406464"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=96057","Source__c":"C0406464","Xref__c":"MEDGEN:96057"},{"URL__c":"https://www.orpha.net/en/disease/detail/222","Source__c":"C0406464; MONDO:0016379; ORPHA:222","Xref__c":"ORPHA:222"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0016379","Source__c":"GARD:0002188","Xref__c":"MONDO:0016379"}],"GARD_Disease_Feature__c":[{"Provided_By__c":"ORPHA:222","HPO_Frequency__c":"Very frequent (80-99%)","Feature__r":{"HPO_Description__c":"A small elevation of the skin containing cloudy or purulent material usually consisting of necrotic inflammatory cells.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0200039","HPO_Synonym__c":"Pimple; Pustula; Pustular lesion; Pustules; Skin pustule; Skin pustules","HPO_Name__c":"Pustule","Feature_System__c":"Skin System; Immune System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:222","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0004552","HPO_Synonym__c":"Cicatricial alopecia","HPO_Name__c":"Scarring alopecia of scalp","Feature_System__c":"Skin System; Musculoskeletal System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:222","HPO_Frequency__c":"Very frequent (80-99%)","Feature__r":{"HPO_Description__c":"An abnormality of the hair.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0001595","HPO_Synonym__c":"Abnormality of the hair; Hair abnormality","HPO_Name__c":"Abnormal hair morphology","Feature_System__c":"Skin System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:222","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"A discontinuity of the skin exhibiting incomplete loss of the epidermis, a lesion that is moist, circumscribed, and usually depressed.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0200041","HPO_Name__c":"Skin erosion","Feature_System__c":"Skin System","HPO_Feature_Type__c":"Symptom"}},{"Provided_By__c":"ORPHA:222","HPO_Frequency__c":"Very frequent (80-99%)","Feature__r":{"HPO_Description__c":"Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0010783","HPO_Synonym__c":"Redness of skin or mucous membrane","HPO_Name__c":"Erythema","Feature_System__c":"Skin System; Cardiovascular System","HPO_Feature_Type__c":"Symptom"}}],"tags":{"Account":["Dermatology"],"Disease Category":["Dermatology"],"Specialist":["Dermatology","Pediatrics"]},"synonyms":[""]}