{"orpha":454710,"name":"Anti-p200 pemphigoid","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, acquired, subepidermal autoimmune bullous disease characterized by polymorphic cutaneous lesions (blisters, urticarial lesions or scars/milia) associated with imunoglubulin G deposition in the basement membrane zone. Lesions are frequently localized on extremities, trunk, palmoplantar and cephalic areas as well as mucous membranes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-454710/","orphanet_url":"https://www.orpha.net/en/disease/detail/454710","names":{"fr":{"name":"Pemphigoïde anti-p200","definition":"Maladie bulleuse auto-immune sous-épidermique rare et acquise, caractérisée par des lésions cutanées polymorphes (vésicules, lésions urticariennes ou cicatrices/milia) associées à un dépôt d'imunoglubuline G au niveau de la membrane basale. Les lésions sont souvent localisées sur les extrémités, le tronc, les régions palmoplantaires et de la tête, ainsi que sur les muqueuses."},"de":{"name":"Anti-p200-Pemphigoid","definition":"Eine erworbene subepidermale bullöse Autoimmunerkrankung, die durch polymorphe Hautläsionen (Blasen, urtikarielle Läsionen oder Narben/Milia) in Verbindung mit Imunoglubulin G-Ablagerungen in der Basalmembranzone gekennzeichnet ist. Die Läsionen sind häufig an den Extremitäten, dem Rumpf, den palmoplantaren und zephalen Bereichen sowie an den Schleimhäuten lokalisiert."},"es":{"name":"Penfigoide anti-p200","definition":"Es una enfermedad ampollosa autoinmune subepidérmica, adquirida y poco frecuente, caracterizada por lesiones cutáneas polimórficas (ampollas, lesiones urticariales o cicatrices/milia) asociadas con el depósito de inmunoglobulina G en la zona de la membrana basal. Las lesiones se localizan más frecuentemente en las extremidades, el tronco y las áreas palmoplantar y cefálica, así como en las mucosas."},"it":{"name":"Pemfigoide anti-p200","definition":"Si tratta di una malattia bollosa subepidermica autoimmune acquisita rara, caratterizzata da lesioni cutanee polimorfe (vesciche, lesioni orticaroidi o cicatrici/milia) associate a depositi di immunoglobuline G nella membrana basale. Spesso le lesioni si localizzano sugli arti, sul torace, sulla zona palmoplantare, sulla testa e sulle mucose."},"nl":{"name":"Anti-p200-pemfigoïd","definition":"Een zeldzame, verworven, subepidermale auto-immune bulleuze ziekte, gekarakteriseerd door polymorfe cutane laesies (blaren, urticariële laesies of littekens/milia) geassocieerd met afzetting van immunoglobuline G in basale membraan. Laesies zijn vaak gesitueerd op ledematen, romp, palmoplantaire en cefale gebieden, alsook slijmvlies."},"pt":{"name":"Penfigoide anti-p200"},"pl":{"name":"Pemfigoid anty-p200"},"cs":{"name":"Anti-p200 pemfigoid"},"uk":{"name":"Анти-p200 пемфігоїд","definition":"A rare, acquired, subepidermal autoimmune bullous disease characterized by polymorphic cutaneous lesions (blisters, urticarial lesions or scars/milia) associated with imunoglubulin G deposition in the basement membrane zone. Lesions are frequently localized on extremities, trunk, palmoplantar and cephalic areas as well as mucous membranes."},"zh":{"name":"抗p-200类天疱疮","definition":"一种罕见的获得性表皮下自身免疫性大疱性疾病，其特征是与基底膜区中的免疫球蛋白G沉积有关的多形性皮肤病变（水疱，荨麻疹或疤痕/纤毛）。病变常位于四肢，躯干，掌趾，头颅以及粘膜。"},"tr":{"name":"Anti-p200 pemfigoid"}},"xrefs":[{"source":"ICD-10","code":"L12.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB41.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"216741864"},{"source":"MONDO","code":"0018688","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681099","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79669,"name":"Autoimmune bullous skin disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":50.0,"area":"Worldwide","status":"Validated","source":"24767733[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_24767733[PMID]"}],"phenotypes":null}