{"orpha":137807,"name":"Primary cutaneous amyloidosis","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["PLCA","Primary localized cutaneous amyloidosis"],"definition":"A rare group of skin diseases characterized histologically by the extracellular accumulation of amyloid deposits in the dermis. Rare forms include lichen amyloidosus, X-linked reticulate pigmentary disorder, primary localized cutaneous nodular amyloidosis, and macular amyloidosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-137807/","orphanet_url":"https://www.orpha.net/en/disease/detail/137807","names":{"fr":{"name":"Amylose cutanée primitive","synonyms":["PLCA"],"definition":"Le terme « amylose cutanée primitive » se réfère à une variété de maladies de la peau caractérisées sur le plan histologique par l'accumulation extracellulaire de dépôts amyloïdes dans le derme. Les formes rares incluent le lichen amyloïde, la pigmentation rétinoïde liée au chromosome X, l'amylose cutanée nodulaire et l'amylose maculeuse."},"de":{"name":"Primäre kutane Amyloidose","synonyms":["Amyloidose, kutane lokalisierte primäre","PLCA"],"definition":"Als Kutane Amyloidose werden verschiedene Hautkrankheiten bezeichnet, die histologisch durch extrazelluläre Anhäufung von Amyloidablagerungen in der Dermis gekennzeichnet sind. Zu den seltenen Formen gehören der Lichen amyloidosus, die X-chromosomale retikuläre Pigmentstörung, die primäre lokalisierte kutane noduläre Amyloidose und die makuläre Amyloidose (s.diese Termini)."},"es":{"name":"Amiloidosis cutánea primaria","synonyms":["Amiloidosis cutánea primaria localizada","PLCA"],"definition":"La amiloidosis cutánea hace referencia a una variedad de enfermedades de la piel que se caracterizan histológicamente por la acumulación extracelular de depósitos amiloides en la dermis. Entre las formas poco comunes se incluyen el liquen amiloide, el trastorno pigmentario reticular ligado al X, la amiloidosis nodular cutánea localizada primaria, y la amiloidosis macular (consulte estos términos)."},"it":{"name":"Amiloidosi cutanea primitiva","synonyms":["Amiloidosi cutanea localizzata primitiva","PLCA"],"definition":"L'amiloidosi cutanea comprende un gruppo di malattie cutanee caratterizzate, dal punto di vista istologico, dall'accumulo extracellulare di depositi di amiloide nel derma. Le forme rare comprendono il lichen amiloidosico, il disturbo reticolare della pigmentazione legato all'X, l'amiloidosi nodulare cutanea localizzata primitiva e l'amiloidosi maculare (si vedano questi termini)."},"nl":{"name":"Primaire cutane amyloïdose","synonyms":["PLCA","Primaire gelokaliseerde cutane amyloïdose"],"definition":"Een verzameling van huidziekten, histologisch gekenmerkt door extracellulaire accumulatie van deposities van amyloïd in dermis. Zeldzame vormen zijn onder meer lichen amyloidosus, X-gebonden reticulaire pigmentstoornis, primaire gelokaliseerde cutane nodulaire amyloïdose, en maculaire amyloïdose."},"pt":{"name":"Amiloidose cutânea"},"pl":{"name":"Pierwotna amyloidoza skórna","synonyms":["Pierwotna zlokalizowana amyloidoza skórna","plCA"]},"cs":{"name":"Primární kožní amyloidóza","synonyms":["PLCA","Primární lokalizovaná kožní amyloidóza"]},"uk":{"name":"Первинний шкірний амілоїдоз","synonyms":["PLCA","Первинний локалізований шкірний амілоїдоз"],"definition":"A rare group of skin diseases characterized histologically by the extracellular accumulation of amyloid deposits in the dermis. Rare forms include lichen amyloidosus, X-linked reticulate pigmentary disorder, primary localized cutaneous nodular amyloidosis, and macular amyloidosis."},"zh":{"name":"原发性皮肤淀粉样变","synonyms":["原发性皮肤淀粉样变病"],"definition":"皮肤淀粉样变性指多种皮肤疾病，其组织学特征是细胞外积累的淀粉样蛋白沉积在真皮中。罕见的类型包括淀粉样变性苔癣、X连锁网状色素性疾病、原发性局部皮肤结节性淀粉样变性和斑状淀粉样变性（见该类词条）。"},"tr":{"name":"Primer kutanöz amiloidoz","synonyms":["PLCA","Primer lokalize kutanöz amiloidoz"]}},"xrefs":[{"source":"ICD-11","code":"5D00.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"113043090"},{"source":"MONDO","code":"0015301","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15301","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C562642","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0268397","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"132","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10011659","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":79381,"name":"Other dermis disorder","classifications":[187]}],"children":[{"orpha":319635,"name":"Amyloidosis cutis dyschromia","type":"Disease"},{"orpha":353220,"name":"Familial primary localized cutaneous amyloidosis","type":"Disease"},{"orpha":49804,"name":"Lichen amyloidosis","type":"Disease"},{"orpha":137814,"name":"Macular amyloidosis","type":"Disease"},{"orpha":137810,"name":"Nodular cutaneous amyloidosis","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":9.8,"area":"Taiwan, Province of China","status":"Validated","source":"21070198[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}