{"orpha":49804,"name":"Lichen amyloidosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Amyloid lichen","Lichen amyloidosus"],"definition":"Lichen amyloidosis is a rare chronic form of cutaneous amyloidosis, a skin disease characterized by the accumulation of amyloid deposits in the dermis, clinically characterized by the development of pruritic, often pigmented, hyperkeratotic papules on trunk and extremities, especially on the shins, and histologically by the deposition of amyloid or amyloid-like proteins in the papillary dermis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-49804/","orphanet_url":"https://www.orpha.net/en/disease/detail/49804","names":{"fr":{"name":"Lichen amyloïde","synonyms":["Amylose cutanée papuleuse"],"definition":"Le lichen amyloïde est une forme chronique et rare d'amylose cutanée , une maladie de la peau caractérisée par l'accumulation de dépôts amyloïdes dans le derme. Sur le plan clinique, la maladie se manifeste par le développement de papules prurigineuses hyperkératotiques et souvent pigmentées sur le tronc et les extrémités, surtout au niveau des tibias. Sur le plan histologique, elle est caractérisée par des dépôts de protéines amyloïdes ou similaires dans le derme papillaire."},"de":{"name":"Lichen amyloidosus","definition":"Der Lichen amyloidosus ist eine seltene chronische Form der kutane Amyloidose (s. dort), einer Hautkrankheit, die histologisch durch die Ablagerung von Amyloid oder amyloid-artigen Proteinen in der papillären Dermis gekennzeichnet ist. Klinische Merkmale sind das Auftreten von Pruritus und hyperkeratotische, oft pigmentierte Papeln an Stamm und Extremitäten, besonders über den Schienbeinen."},"es":{"name":"Amiloidosis liquenoide","synonyms":["Liquen amiloide"],"definition":"El liquen amiloide es una forma crónica poco frecuente de amiloidosis cutánea (consulte este término), una enfermedad de la piel por acumulación de depósitos amiloides en la dermis, y se caracteriza clínicamente por el desarrollo de pápulas pruriginosas, a menudo pigmentadas, hiperqueratóticas en tronco y extremidades, especialmente en las espinillas, e histológicamente por el depósito de proteínas amiloides o de tipo amiloide en la dermis papilar."},"it":{"name":"Lichen - amiloidosi","synonyms":["Lichen amiloide","Lichen amiloidosico"],"definition":"Il lichen amiloidosico è una forma cronica rara dell'amiloidosi cutanea (si veda questo termine), una malattia cutanea caratterizzata dall'accumulo di depositi di amiloide nel derma, caratterizzata clinicamente dallo sviluppo di papule ipercheratosiche pruriginose, spesso pigmentate, sul tronco e sulle estremità, in particolare, sulle creste tibiali e, dal punto di vista istologico, dal deposito di proteine amiloidi o simil-amiloidi nel derma papillare."},"nl":{"name":"Lichen amyloidosus","synonyms":["Amyloidosis cutis papulosa","Lichen amyloïdose","Papuleuze amyloïdose"],"definition":"Een zeldzame chronische vorm van cutane amyloïdose, een huidziekte gekenmerkt door accumulatie van afzettingen van amyloïd in dermis, klinisch gekenmerkt door ontwikkeling van pruritische, vaak gepigmenteerde, hyperkeratotische papels op romp en extremiteiten, vooral schenen, en histologisch gekenmerkt door depositie van amyloïd of amyloïd-achtige proteïnen in stratum papillare."},"pt":{"name":"Líquen amiloidótico"},"pl":{"name":"Liszaj amyloidowy","synonyms":["Liszaj amyloidowy","Liszaj amyloidowy"]},"cs":{"name":"Lichen amyloidosus","synonyms":["Amyloidní lichen"]},"uk":{"name":"Лишайний амілоїдоз","synonyms":["Амілоїдний лишай","Амілоїдозний лишай"],"definition":"Lichen amyloidosis is a rare chronic form of cutaneous amyloidosis (see this term), a skin disease characterized by the accumulation of amyloid deposits in the dermis, clinically characterized by the development of pruritic, often pigmented, hyperkeratotic papules on trunk and extremities, especially on the shins, and histologically by the deposition of amyloid or amyloid-like proteins in the papillary dermis."},"zh":{"name":"苔藓样淀粉样变病","synonyms":["苔藓样淀粉样变病"]},"tr":{"name":"Liken amiloidoz","synonyms":["Amiloid liken","Liken amiloidozu"]}},"xrefs":[{"source":"ICD-10","code":"E85.4+","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-10","code":"L99.0*","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"5D00.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1889504911"},{"source":"MONDO","code":"0018856","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5779561","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":137807,"name":"Primary cutaneous amyloidosis","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult"],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0200034","label":"Papule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Very frequent (99-80%)","criteria":null}]}}