{"orpha":83453,"name":"Vulvovaginal gingival syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, non-malformative vulvovaginal disease characterized by a combination of erosive or desquamative lichen planus (LP) of vulval, vaginal and gingival mucosae, with a high propensity for scarring and stricture formation. Additional sites of involvement are frequently observed (in particular, tongue, buccal mucosae, skin and perianal LP). Patients may be asymptomatic or, more commonly, present with pain, burning, discomfort and bleeding, dyspareunia, and seropurulent vaginal discharge.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83453/","orphanet_url":"https://www.orpha.net/en/disease/detail/83453","names":{"fr":{"name":"Syndrome vulvo-vaginal-gingival","definition":"Maladie vulvo-vaginale rare autre que malformative caractérisée par une association de lichen plan (LP) érosif ou desquamatif des muqueuses vulvaires, vaginales et gingivales, et par une forte tendance à la formation de cicatrices et de sténoses. Le LP peut affecter d'autres régions du corps (en particulier la langue, les muqueuses buccales, la peau et la zone périanale). Les patients peuvent être asymptomatiques ou, plus généralement, manifester des douleurs, des brûlures, une gêne, des saignements, une dyspareunie et des pertes vaginales séropurulentes."},"de":{"name":"Vulvovagina-Gingiva-Syndrom","definition":"Eine seltene vulvovaginale Erkrankung, die durch eine Kombination aus erosivem oder desquamativem Lichen planus (LP) der vulvalen, vaginalen und gingivalen Schleimhäute gekennzeichnet ist, mit einer hohen Neigung zur Narben- und Strikturbildung. Häufig werden noch weitere Befallsstellen beobachtet (insbesondere Zunge, Wangenschleimhaut, Haut und perianaler LP). Die Patienten können asymptomatisch sein oder, was häufiger der Fall ist, mit Schmerzen, Brennen, Unbehagen und Blutungen, Dyspareunie und seropurulentem Vaginalausfluss auftreten."},"es":{"name":"Síndrome vulvovaginal-gingival","definition":"Es una enfermedad vulvovaginal no malformativa poco frecuente caracterizada por una combinación de liquen plano (LP) erosivo o descamativo de las mucosas vulvar, vaginal y gingival, con una propensión elevada a la formación de cicatrices y estenosis. Con frecuencia se observan sitios adicionales de afectación (en particular, LP de la lengua, mucosas bucales, piel y zona perianal). Los pacientes pueden estar asintomáticos o, con mayor frecuencia, presentar dolor, ardor, malestar y sangrado, dispareunia y flujo vaginal seropurulento."},"it":{"name":"Sindrome vulvovaginale-gengivale","definition":"Si tratta di una malattia vulvovaginale rara non malformativa, caratterizzata da lichen planus (LP) erosivo o desquamativo delle mucose della vulva, della vagina e delle gengive, associata a spiccata predisposizione alla formazione di cicatrici e stenosi. La malattia può interessare anche altre zone (in particolare LP della lingua, delle mucose della bocca, della cute e della zona perianale). Le pazienti possono essere asintomatiche, ma di solito presentano dolore, bruciore, disagio, emorragie, dispareunia e perdite vaginali sieropurulente."},"nl":{"name":"Vulvovaginaal-gingivaal syndroom","definition":"Een zeldzame, niet-malformatieve vulvovaginale aandoening, gekarakteriseerd door een combinatie van erosieve of desquamatieve lichen planus (LP) van slijmvlies van vulva, vagina en gingiva, met neiging tot strictuur- en littekenvorming. Vaak wordt bijkomende betrokkenheid van andere locaties waargenomen (vooral LP van tong, wangslijmvlies, huid en perianale regio). Patiënten kunnen asymptomatisch zijn, maar presenteren zich meestal met pijn, brandend gevoel, ongemak en bloeding, dyspareunie, en seropurulente vaginale afscheiding."},"pt":{"name":"Síndrome vulvo-vaginal - gengival"},"pl":{"name":"Zespół sromowo-waginalno-dziąsłowy"},"cs":{"name":"Vulvovaginogingivální syndrom"},"uk":{"name":"Вульвовагінальний ясенний синдром","definition":"A rare, non-malformative vulvovaginal disease characterized by a combination of erosive or desquamative lichen planus (LP) of vulval, vaginal and gingival mucosae, with a high propensity for scarring and stricture formation. Additional sites of involvement are frequently observed (in particular, tongue, buccal mucosae, skin and perianal LP). Patients may be asymptomatic or, more commonly, present with pain, burning, discomfort and bleeding, dyspareunia, and seropurulent vaginal discharge."},"zh":{"name":"外阴阴道牙龈综合征"},"tr":{"name":"Vulvovajinal dişeti sendromu"}},"xrefs":[{"source":"ICD-10","code":"L43.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EA91.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"997964040"},{"source":"MONDO","code":"0019370","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3873472","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":96344,"name":"Rare gynecologic or obstetric disease"},"parents":[{"orpha":254373,"name":"Rare mucosal lichen planus","classifications":[187]},{"orpha":180205,"name":"Rare non-malformative uterovaginal or vulvovaginal disease","classifications":[205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":380.0,"area":"Worldwide","status":"Validated","source":"20373919[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-08-05","source":"21763554[PMID]_20373919[PMID]","terms":[{"hpo":"HP:0000230","label":"Gingivitis","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0000055","label":"Abnormality of female external genitalia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011118","label":"Abnormality of tumor necrosis factor secretion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025092","label":"Epidermal acanthosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012537","label":"Food intolerance","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100725","label":"Lichenification","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001036","label":"Parakeratosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001807","label":"Ridged nail","frequency":"Occasional (29-5%)","criteria":null}]}}