{"Name":"Infective dermatitis associated with HTLV-1","DiseaseID__c":"GARD:0021135","id":21135,"encodedName":"infective-dermatitis-associated-with-htlv-1","IsDeleted":false,"Disease_Name_Full__c":"Infective dermatitis associated with HTLV-1","Xref_IDs__c":"C129260; C4274291; MEDGEN:895843; MONDO:0017326; ORPHA:289347","USA_Estimate__c":null,"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":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":4,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0017326","Disease_Description__c":"Infective dermatitis associated with HTLV-1 is a rare and severe chronic disease characterized by recurrent chronic eczema (with erythematous, scaly and crusted lesions) mainly affecting seborrheic areas (e.g. scalp, forehead, eyelids, paranasal and periauricular skin, neck, axillae, and groin), a generalized fine papular rash, chronic nasal discharge with crusting of the anterior nares, and non-virulent <i>Staphylococcus aureus</i> or beta-hemolytic <i>Streptococcus</i> infections, thought to be a result of HTLV-1-induced immunosuppression. Lymphadenopathy, anemia, mild to moderate pruritus and increased incidence of other infections (e.g. crusted scabies) have also been reported in some patients. Patients may subsequently develop other HTLV-1 associated conditions such as adult T-cell leukemia/lymphoma and tropical spastic paraparesis (see these terms).","GARD_Name__c":"Infective dermatitis associated with HTLV-1","GARD_Synonym__c":"idh; idh gene family; infective dermatitis associated with human t-lymphotropic virus type 1; infective dermatitis associated with human t-lymphotropic virus type i; isocitrate dehydrogenase gene family","Curated_Disease_Description_Source__c":"ORPHA:289347","Curated_Disease_Description__c":"Infective dermatitis associated with HTLV-1 is a rare and severe chronic disease characterized by recurrent chronic eczema (with erythematous, scaly and crusted lesions) mainly affecting seborrheic areas (e.g., scalp, forehead, eyelids, paranasal and periauricular skin, neck, axillae, and groin), a generalized fine papular rash, chronic nasal discharge with crusting of the anterior nares, and non-virulent <i>Staphylococcus aureus</i> or beta-hemolytic <i>Streptococcus</i> infections, thought to be a result of HTLV-1-induced immunosuppression. Lymphadenopathy, anemia, mild to moderate pruritus and increased incidence of other infections (e.g., crusted scabies) have also been reported in some patients. Patients may subsequently develop other HTLV-1 associated conditions such as adult T-cell leukemia/lymphoma and tropical spastic paraparesis.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":"as a Child","SourceID__c":"ORPHA:289347","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0017326","ORPHANET_ID__c":"ORPHA:289347","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Dermatitis infecciosa asociada al htlv-1","Spanish_Description_Source__c":"ORPHA:289347","Spanish_Description__c":"Es una enfermedad grave, poco frecuente y crónica caracterizada por un eccema crónico recurrente (lesiones eritematosas, descamativas y costrosas) que afecta principalmente las áreas seborreicas (p. ej., cuero cabelludo, frente, párpados, región paranasal y preauricular, cuello, axilas e ingles), una erupción papular fina generalizada, exudación nasal crónica con formación de costras en las narinas e infecciones por <i>Staphylococcus aureus</i> o por <i>Streptococcus </i> beta-hemolítico, considerada como una consecuencia de la inmunosupresión inducida por HTLV-1. En algunos pacientes, también se han descrito poliadenopatías, anemia, prurito de intensidad variable y una mayor incidencia de otros procesos infecciosos (p. ej., sarna costrosa). Los individuos afectos pueden desarrollar posteriormente otras procesos asociados a HTLV-1, como un linfoma / leucemia de células T del adulto y una paraparesia espástica tropical.","Spanish_Disease_Name__c":"dermatitis infecciosa asociada al htlv-1","Spanish_GARD_Synonym__c":"dermatitis infecciosa asociada al virus linfotrópico de células t humanas tipo 1; dermatitis infecciosa asociada al virus linfotrópico de células t humanas tipo i; idh","Category_Linearization__c":"ORPHA:68416","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":"Infective dermatitis associated with HTLV-1 is a rare and severe chronic disease characterized by recurrent chronic eczema (with erythematous, scaly and crusted lesions) mainly affecting seborrheic areas (e.g., scalp, forehead, eyelids, paranasal and periauricular skin, neck, axillae, and groin), a generalized fine papular rash, chronic nasal discharge with crusting of the anterior nares, and non-virulent <i>Staphylococcus aureus</i> or beta-hemolytic <i>Streptococcus</i> infections, thought to be a result of HTLV-1-induced immunosuppression. Lymphadenopathy, anemia, mild to moderate pruritus and increased incidence of other infections (e.g., crusted scabies) have also been reported in some patients. Patients may subsequently develop other HTLV-1 associated conditions such as adult T-cell leukemia/lymphoma and tropical spastic paraparesis.","Curated_Disease_Description_Source__c":"ORPHA:289347","GARD_Synonym__c":"idh; idh gene family; infective dermatitis associated with human t-lymphotropic virus type 1; infective dermatitis associated with human t-lymphotropic virus type i; isocitrate dehydrogenase gene family","Name":"Infective dermatitis associated with HTLV-1","estimateUsa":""}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Infectious Disease","Tag_Category__c":"Disease Category;Specialist","category_description":"Infectious diseases are caused by bacteria, viruses, fungi, or parasites entering the body, multiplying, and spreading illness.","curated_tag_name":"Infectious 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":"Rare Viral Disease","Tag_Category__c":"Cause","curated_tag_name":"Viral Infectious Diseases"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Childhood","Provided_By__c":"ORPHA:289347"}],"External_Identifier_Disease__c":[{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C4274291","Source__c":"C4274291","Xref__c":"C4274291"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=895843","Source__c":"C4274291","Xref__c":"MEDGEN:895843"},{"URL__c":"https://www.orpha.net/en/disease/detail/289347","Source__c":"C4274291; MONDO:0017326; ORPHA:289347","Xref__c":"ORPHA:289347"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C129260","Source__c":"MONDO:0017326","Xref__c":"C129260"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0017326","Source__c":"GARD:0021135","Xref__c":"MONDO:0017326"}],"tags":{"Disease Category":["Infectious Disease","Dermatology"],"Specialist":["Infectious Disease","Dermatology","Pediatrics"],"Account":["Dermatology"],"Cause":["Rare Viral Disease"]},"synonyms":["idh"," idh gene family"," infective dermatitis associated with human t-lymphotropic virus type 1"," infective dermatitis associated with human t-lymphotropic virus type i"," isocitrate dehydrogenase gene family"]}