{"orpha":2221,"name":"Acquired hypertrichosis lanuginosa","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare cutaneous paraneoplastic disease characterized by the presence of excessive lanugo-type hair on the glabrous skin of face, neck, trunk and limbs that can be associated with additional clinical features such as burning glossitis, papillary hypertrophy of the tongue, diarrhea, dysgeusia, and/or weight loss. It is associated with lymphoma or cancer of the gastrointestinal system, urinary tract, lung, breast, uterus or ovary.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2221/","orphanet_url":"https://www.orpha.net/en/disease/detail/2221","names":{"fr":{"name":"Hypertrichose lanugineuse acquise","definition":"L'hypertrichose lanugineuse acquise est une affection paranéoplasique cutanée rare caractérisée par la présence excessive de poils de type lanugo sur la peau glabre du visage, du cou, du tronc et des membres, pouvant être associée à des manifestations cliniques additionnelles telles qu'une glossite douloureuse, une hypertrophie papillaire de la langue, une diarrhée, une dysgueusie et/ou une perte de poids. Elle est associée au développement d'un lymphome ou d'un cancer digestif, urinaire, pulmonaire, du sein, utérin ou ovarien."},"de":{"name":"Hypertrichosis lanuginosa, erworbene","definition":"Die Erworbene Hypertrichosis lanuginosa ist eine seltene, paraneoplastische Hautkrankheit mit exzessiver Lanugo-Behaarung der haarlosen Bereiche des Gesichtes, des Stamms und der Gliedmassen und möglichen weiteren klinischen Symptomen, darunter 'brennende' Glossitis, Hypertrophie der Zungenpapillen, Diarrhoe, Dysgeusie (Schmeckstörung) und/oder Gewichtsverlust. Die Krankheit ist assoziiert mit Lymphom oder Krebs in unterschiedlichen Lokalisationen (Magen-Darm-Trakt, Harnwege, Lunge, Brust, Uterus oder Ovar)."},"es":{"name":"Hipertricosis lanuginosa adquirida","definition":"Es una enfermedad paraneoplásica cutánea poco frecuente que se caracteriza por la presencia excesiva de pelo tipo lanugo en la piel glabra de la cara, cuello, tronco y extremidades que puede estar asociada con rasgos clínicos adicionales como glositis ardiente, hipertrofia papilar de la lengua, diarrea, disgeusia, y/o pérdida de peso. Está asociada a linfoma o cáncer en el sistema gastrointestinal, tracto urinario, pulmón, mama, útero u ovario."},"it":{"name":"Ipertricosi lanuginosa acquisita","definition":"L'ipertricosi lanuginosa acquisita è una malattia rara paraneoplastica della cute, caratterizzata dalla presenza di un eccesso di capelli lanuginosi sulla parte glabra del viso, del collo, del tronco e degli arti, che può associarsi ad altre caratteristiche cliniche, come la glossite bruciante, l'ipertrofia papillare della lingua, la diarrea, la disgeusia, e/o la perdita di peso. Si associa al linfoma o al tumore dell'apparato gastrointestinale, del tratto urinario, del polmone, della mammella, dell'utero o dell'ovaio."},"nl":{"name":"Verworven hypertrichosis lanuginosa","definition":"Een zeldzame, cutane, paraneoplastische ziekte, gekarakteriseerd door de aanwezigheid van excessief lanugohaar op de haarloze huid van gelaat, nek, romp en ledematen dat geassocieerd kan zijn met bijkomende klinische kenmerken zoals glossitis met brandend gevoel, papillaire hypertrofie van de tong, diarree, dysgeusie, en/of gewichtsverlies. Het is geassocieerd met lymfoom of kanker van gastro-intestinaal systeem, urinewegstelsel, long, borst, uterus of ovarium."},"pt":{"name":"Hipertricose lanuginosa adquirida"},"pl":{"name":"Nabyte nadmierne owłosienie typu płodowego"},"cs":{"name":"Získaná lanuginózní hypertrichóza"},"uk":{"name":"Набутий гіпертрихоз ланугіноз","definition":"A rare cutaneous paraneoplastic disease characterized by the presence of excessive lanugo-type hair on the glabrous skin of face, neck, trunk and limbs that can be associated with additional clinical features such as burning glossitis, papillary hypertrophy of the tongue, diarrhea, dysgeusia, and/or weight loss. It is associated with lymphoma or cancer of the gastrointestinal system, urinary tract, lung, breast, uterus or ovary."},"zh":{"name":"获得性胎毛增多症"},"tr":{"name":"Edinsel hipertrikoz lanuginoza"}},"xrefs":[{"source":"ICD-10","code":"L68.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EL10","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1885858920"},{"source":"MONDO","code":"0016380","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0343072","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2864","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79365,"name":"Rare disorder with hypertrichosis","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":60.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-09-27","source":null,"terms":[{"hpo":"HP:0000534","label":"Abnormal eyebrow morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000492","label":"Abnormal eyelid morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002213","label":"Fine hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002230","label":"Generalized hirsutism","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005599","label":"Hypopigmentation of hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002664","label":"Neoplasm","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000206","label":"Glossitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000158","label":"Macroglossia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000956","label":"Acanthosis nigricans","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002028","label":"Chronic diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008064","label":"Ichthyosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100013","label":"Neoplasm of the breast","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100606","label":"Neoplasm of the respiratory system","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100615","label":"Ovarian neoplasm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001072","label":"Thickened skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null}]}}