{"orpha":228379,"name":"Virus-associated trichodysplasia spinulosa","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cyclosporine-induced folliculodystrophy","Pilomatrix dysplasia","TS","Trichodysplasia spinulosa","VATS"],"definition":"Virus-associated trichodysplasia spinulosa is a rare infectious skin disease characterized by the development of follicular papules with keratin spicules in various parts of the body, predominantly in the face (e.g. nose, eyebrows, auricles), that is due to polyomavirus infection in immunocompromized patients.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-228379/","orphanet_url":"https://www.orpha.net/en/disease/detail/228379","names":{"fr":{"name":"Trichodysplasie spinulosa","synonyms":["Dystrophie du follicule pileux induite par la cyclosporine","TS","VATS"],"definition":"La trichodysplasie spinulosa est une maladie infectieuse cutanée rare caractérisée par le développement de papules folliculaires avec des spicules de kératine à plusieurs endroits du corps, surtout au niveau du visage (nez, sourcils, pavillon de l'oreille). Elle est due à une infection à polyomavirus survenant chez des patients immunodéprimés."},"de":{"name":"Trichodysplasia spinulosa, Virus-assoziierte","synonyms":["Cyclosporin-induzierte Follikulotropie","Pilomatrix-Dysplasie","TS","Trichodysplasia spinulosa","VATS"],"definition":"Die Virus-assoziierte Trichodysplasia spinulosa ist eine seltene Infektionskrankheit der Haut. Sie ist gekennzeichnet durch die Bildung follikulärer Papeln mit Keratinnadeln in verschiedenen Bereichen des Körpers, vor allem aber im Gesicht (Nase, Augenbrauen, Ohren). Ursache ist eine Infektion mit Polyomvirus bei immungeschwächten Patienten."},"es":{"name":"Tricodisplasia espinulosa asociada a virus","synonyms":["Displasia pilomatrix","Distrofia folicular inducida por ciclosporina","TS","Tricodisplasia espinulosa","VATS"],"definition":"La tricodisplasia espinulosa asociada a virus es una enfermedad infecciosa rara de la piel que se caracteriza por el desarrollo de pápulas foliculares con espículas de queratina en varias partes del cuerpo, predominantemente en la cara (p.e. la nariz, cejas, aurículas), que es debido a una infección por poliomavirus en pacientes inmunocomprometidos."},"it":{"name":"Tricodisplasia spinulosa associata a virus","synonyms":["Displasia della pilomatrice","Distrofia follicolare indotta dalla ciclosporina","TS","Tricodisplasia spinulosa","VATS"],"definition":"La tricodisplasia spinulosa associata ai virus è una malattia cutanea infettiva rara, caratterizzata dallo sviluppo di papule follicolari con spicole cheratiniche in diverse parti del corpo, prevalentemente sulla faccia (naso, sopracciglia, padiglioni auricolari), dovuta a poliomavirosi nei pazienti immunocompromessi."},"nl":{"name":"Virusgeassocieerde trichodysplasia spinulosa","synonyms":["Cyclosporinegeïnduceerde folliculodystrofie","Pilomatrixdysplasie","TS","Trichodysplasia spinulosa","VATS"],"definition":"Een zeldzame infectieuze aandoening van huid, gekenmerkt door ontwikkeling van folliculaire papels met uitsteeksels van keratine op verschillende delen van het lichaam, vooral aangezicht (neus, wenkbrauwen, oorschelpen), als gevolg van infectie door polyomavirus bij immuungecompromitteerde patiënten."},"pt":{"name":"Tricodisplasia espinulosa associada a vírus"},"pl":{"name":"Trichodysplazja spowodowana zakażeniem wirusowym","synonyms":["Dysplazja macierzy włosa","TS","VATS"]},"cs":{"name":"S viry asociovaná trichodysplasia spinulosa","synonyms":["Cyklosporinem indukovaná folikulodystrofie","Pilomatrix dysplasia"]},"uk":{"name":"Вірус-асоційована спінульозна триходисплазія","synonyms":["Фолікулодистрофія, спричинена циклоспорином","Піломатріксна дисплазія","Триходисплазія спінульозна","TS","VATS"],"definition":"Virus-associated trichodysplasia spinulosa is a rare infectious skin disease characterized by the development of follicular papules with keratin spicules in various parts of the body, predominantly in the face (e.g. nose, eyebrows, auricles), that is due to polyomavirus infection in immunocompromized patients."},"zh":{"name":"病毒相关性棘状毛发育不良","synonyms":["环孢菌素诱发毛囊营养不良"],"definition":"病毒相关的毛发发育不良有棘目一种罕见的传染性皮肤病，其特征是在身体的各个部位，主要是在面部（如鼻子、眉毛、耳廓）出现带有角蛋白针状物的滤泡性丘疹，这是由免疫低下患者被多瘤病毒感染所致。"},"tr":{"name":"Virüs -ilişkili trikodisplazi spinuloza","synonyms":["TS","KDV","Pilomatriks displazisi","Trikodisplazi spinulosa","Siklosporin-kaynaklı folikülodistrofi"]}},"xrefs":[{"source":"ICD-10","code":"L08.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"ED5Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1539193990"},{"source":"MONDO","code":"0016455","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3267126","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10072358","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":90077,"name":"Other acquired skin disease","classifications":[187]},{"orpha":163585,"name":"Rare viral disease","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":7.0,"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":null}