{"orpha":692256,"name":"Isolated anogenital granulomatosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["AGG"],"definition":"A rare urogenital disease characterized by the presence of granulomas in the genital or anoperineal area presenting with painless swelling, inflammation, ulcers and lymphoedema. It is usually associated with a history of recurrent perianal or genital lesions.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-692256/","orphanet_url":"https://www.orpha.net/en/disease/detail/692256","names":{"fr":{"name":"Granulomatose anogénitale isolée","synonyms":["GAG"],"definition":"Maladie urogénitale rare caractérisée par la présence de granulomes dans la région génitale ou anopérinéale, se manifestant par un gonflement indolore, une inflammation, des ulcères et un lymphoedème. Elle est généralement associée à des antécédents de lésions périanales ou génitales récurrentes."},"de":{"name":"Isolierte anogenitale Granulomatose","synonyms":["AGG"],"definition":"Eine seltene urogenitale Krankheit, die durch Granulome im Genital- oder Anoperinealbereich gekennzeichnet ist und sich durch schmerzlose Schwellungen, Entzündungen, Geschwüre und Lymphödeme äußert. Sie geht in der Regel mit wiederkehrenden perianalen oder genitalen Läsionen einher."},"es":{"name":"Granulomatosis anogenital aislada","synonyms":["AGG"],"definition":"Es una enfermedad urogenital poco frecuente caracterizada por la presencia de granulomas en la zona genital o anoperineal, que se presenta con hinchazón indolora, inflamación, úlceras y linfedema. Suele asociarse con antecedentes de lesiones perianales o genitales recurrentes."},"it":{"name":"Granulomatosi anogenitale isolata","synonyms":["AGG"]},"nl":{"name":"Geïsoleerde anogenitale granulomatose","synonyms":["AGG"],"definition":"Een zeldzame urogenitale aandoening, gekenmerkt door de aanwezigheid van granulomen in het genitale of anoperineale gebied die zich presenteren met pijnloze zwelling, inflammatie, ulcera en lymfoedeem. Het is meestal geassocieerd met een voorgeschiedenis van recidiverende perianale of genitale laesies."},"pt":{"name":"Granulomatose anogenital isolada","synonyms":["AGG"]},"pl":{"name":"Izolowana ziarniniakowatość odbytu i narządów płciowych","synonyms":["AGG"]},"cs":{"name":"Izolovaná anogenitální granulomatóza","synonyms":["AGG"]}},"xrefs":[{"source":"ICD-10","code":"L29.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EC90.5","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":101433,"name":"Rare urogenital disease"},"parents":[{"orpha":79381,"name":"Other dermis disorder","classifications":[187]},{"orpha":180205,"name":"Rare non-malformative uterovaginal or vulvovaginal disease","classifications":[205]},{"orpha":101433,"name":"Rare urogenital disease","classifications":[185]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}