{"orpha":228264,"name":"Papular elastorrhexis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, acquired, dermis elastic tissue disorder characterized by multiple, asymptomatic, firm, well-demarcated, nonfollicular, hypopigmented or skin-colored papules, with a diameter of less than 1 cm, distributed symmetrically over trunk and/or proximal limbs (rarely, head, neck, shoulders, armpits, thighs), with no extracutaneous manifestations. Histopathology typically reveals decreased and fragmented elastic fibers, thickened and/or homogenized collagen bundles and, in some, a mild, perivascular, lymphocytic infiltrate in the dermis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-228264/","orphanet_url":"https://www.orpha.net/en/disease/detail/228264","names":{"fr":{"name":"Elastorrhexie papuleuse","definition":"Anomalie acquise de l'élasticité du derme rare, caractérisée par de multiples papules asymptomatiques, fermes, bien délimitées, non folliculaires, hypopigmentées ou de couleur chair, d'un diamètre inférieur à 1 cm, réparties de manière symétrique sur le tronc et/ou la partie proximale des membres (rarement sur la tête, le cou, les épaules, les aisselles, les cuisses), en l'absence de manifestations extracutanées. L'examen histopathologue révèle généralement en nombre réduit de fibres élastiques et fragmentées, des faisceaux de collagène épaissis et/ou homogénéisés et, dans certains cas, un léger infiltrat lymphocytaire périvasculaire dans le derme."},"de":{"name":"Elastorrhexis, papulöse","synonyms":["Naevus anelasticus, disseminierter","Nevus anelasticus"],"definition":"Eine seltene, erworbene Störung des elastischen Gewebes der Dermis, die durch multiple, asymptomatische, feste, gut abgegrenzte, nicht-follikuläre, hypopigmentierte oder hautfarbene Papeln mit einem Durchmesser von weniger als 1 cm gekennzeichnet ist, die symmetrisch über den Rumpf und/oder die proximalen Gliedmaßen (selten Kopf, Nacken, Schultern, Achselhöhlen, Oberschenkel) verteilt sind und keine extrakutanen Manifestationen aufweisen. Die Histopathologie zeigt typischerweise verminderte und fragmentierte elastische Fasern, verdickte und/oder homogenisierte Kollagenbündel und in einigen Fällen ein leichtes, perivaskuläres, lymphozytäres Infiltrat in der Dermis."},"es":{"name":"Elastorrexis papular","definition":"Es un trastorno adquirido y poco frecuente del tejido elástico de la dermis caracterizado por múltiples pápulas asintomáticas, firmes, bien delimitadas, no foliculares, hipopigmentadas o de color carne, con un diámetro inferior a 1 cm, distribuidas simétricamente sobre el tronco y/o en la parte proximal de las extremidades (excepcionalmente en la cabeza, el cuello, los hombros, las axilas, y los muslos), sin manifestaciones extra-cutáneas. Por lo general, la histopatología revela una disminución y fragmentación de las fibras elásticas, haces de colágeno engrosados y/u homogeneizados y, en algunos casos, un ligero infiltrado linfocítico perivascular en la dermis."},"it":{"name":"Elastoressi papulosa","synonyms":["Collagenoma eruttivo","Nevo anelastico","Nevo anelastico disseminato"],"definition":"È una malattia acquisita rara del tessuto elastico del derma, caratterizzata da papule multiple asintomatiche, solide, ben delineate, non follicolari, ipopigmentate o dello stesso colore della cute, con un diametro inferiore ad un centimetro, distribuite in modo simmetrico sul tronco e/o sulla porzione prossimale degli arti (raramente sulla testa, sul collo, sulle spalle, sulle ascelle e sulle cosce), in assenza di sintomi extracutanei. L'esame istologico evidenzia la diminuzione e la frammentazione delle fibre elastiche, bande di collagene ispessite e/o omogenee e, occasionalmente, un lieve infiltrato linfocitico perivascolare nel derma."},"nl":{"name":"Papula-elastorrhexis","definition":"Een zeldzame, verworven stoornis van elastisch weefsel van de dermis, gekarakteriseerd door multipele, asymptomatische, stevige, goed begrensde, niet-folliculaire, gehypopigmenteerde of huidkleurige papels, met een diameter van minder dan 1 cm, symmetrisch verdeeld over de romp en/of proximale ledematen (zelden op hoofd, nek/hals, schouders, oksels, dijen), en zonder extracutane manifestaties. Histopathologie toont doorgaans verminderde en gefragmenteerde elastische vezels, verdikte en/of gehomogeniseerde collageenbundels en, bij sommigen, een mild, perivasculair, lymfocytisch infiltraat in de dermis."},"pt":{"name":"Elastorrhexis papular"},"pl":{"name":"Grudkowa fragmentacja włókien elastyny","synonyms":["Eruptive collagenoma Wyrzutowa kolagenoma","Rozsiane znamię ze zwyrodnieniem włókien sprężystych","Znamię ze zwyrodnieniem włókien sprężystych"]},"cs":{"name":"Papulární elastorrhexis","synonyms":["Diseminovaný nevus anelasticus","Eruptivní kolagenom","Nevus anelasticus"]},"uk":{"name":"Папульозний еласторексис","definition":"A rare, acquired, dermis elastic tissue disorder characterized by multiple, asymptomatic, firm, well-demarcated, nonfollicular, hypopigmented or skin-colored papules, with a diameter of less than 1 cm, distributed symmetrically over trunk and/or proximal limbs (rarely, head, neck, shoulders, armpits, thighs), with no extracutaneous manifestations. Histopathology typically reveals decreased and fragmented elastic fibers, thickened and/or homogenized collagen bundles and, in some, a mild, perivascular, lymphocytic infiltrate in the dermis."},"zh":{"name":"丘疹性弹性组织破裂","definition":"一种罕见的获得性真皮弹性组织疾病，特征为多发、无症状、坚硬、界限清楚、非滤泡性色素减退或肤色丘疹，直径小于1cm，对称分布于躯干和/或近端肢体（少见与头、颈、肩、腋窝、大腿），无皮肤以外表现。组织病理学通常显示出真皮中弹性纤维减少和断裂，胶原束增厚和/或均质化，某些情况下可见真皮中有轻度血管周围淋巴细胞浸润。"},"tr":{"name":"Papüler elastoreksis"}},"xrefs":[{"source":"ICD-10","code":"L98.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE41.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1231672259"},{"source":"MONDO","code":"0016443","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680907","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":228221,"name":"Acquired dermis elastic tissue disorder with decreased elastic tissue","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}