{"orpha":221142,"name":"Confetti-like macular atrophy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, acquired, dermis elastic tissue disorder with decreased elastic tissue characterized by multiple, asymptomatic, well demarcated, flat, hypopigmented atrophic macular skin lesions distributed over upper trunk and proximal upper limbs. Histopathological examination reveals atrophic epidermis with decreased basal pigmentation, perivascular mononuclear infiltration in the upper dermis, and disorganized, hyalinized, coarse collagen bundles, and variable loss of elastic fibers in the dermis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-221142/","orphanet_url":"https://www.orpha.net/en/disease/detail/221142","names":{"fr":{"name":"Atrophie maculaire type confetti-like","definition":"Anomalie acquise rare de l'élasticité du derme avec perte de tissus élastique, caractérisée par de multiples lésions cutanées maculaires atrophiques, asymptomatiques, bien délimitées, planes et hypopigmentées, affectant la partie supérieure du tronc et la zone proximale des membres supérieurs. L'examen histopathologique révèle un épiderme atrophié et une diminution de la pigmentation de la couche basale, une infiltration périvasculaire du derme supérieur par des cellules mononucléées, et des faisceaux de collagène désorganisés, hyalinisés et grossiers, ainsi qu'une perte variable de fibres élastiques dans le derme."},"de":{"name":"Anetodermie, konfettiartige","definition":"Eine seltene, erworbene Störung des elastischen Gewebes der Dermis mit vermindertem elastischen Gewebe, die durch multiple, asymptomatische, gut abgegrenzte, flache, hypopigmentierte atrophische makuläre Hautläsionen gekennzeichnet ist, die über den oberen Rumpf und die proximalen oberen Gliedmaßen verteilt sind. Die histopathologische Untersuchung zeigt eine atrophische Epidermis mit verminderter Basalpigmentierung, perivaskuläre mononukleäre Infiltration in der oberen Dermis und desorganisierte, hyalinisierte, grobe Kollagenbündel sowie einen variablen Verlust an elastischen Fasern in der Dermis."},"es":{"name":"Atrofia macular en confetti","definition":"Es un trastorno adquirido y poco frecuente de disminución del tejido elástico de la dermis caracterizado por múltiples lesiones cutáneas maculares atróficas, hipopigmentadas, planas, bien delimitadas y asintomáticas distribuidas en el tronco superior y en la zona proximal de las extremidades superiores. El examen histopatológico revela atrofia epidérmica con disminución de la pigmentación basal, infiltrado mononuclear perivascular en la dermis superior y haces gruesos de fibras de colágeno desorganizados y hialinizados con una pérdida variable de fibras elásticas dérmicas."},"it":{"name":"Atrofia maculare simil-coriandoli","definition":"È una malattia acquisita rara del tessuto elastico del derma, associata ad una riduzione del tessuto elastico, caratterizzata da lesioni cutanee maculari multiple asintomatiche, ben delimitate, piatte, ipopigmentate e atrofiche, distribuite sulla porzione superiore del tronco e sulla porzione prossimale degli arti superiori. Gli esami istologici evidenziano un'atrofia dell'epidermide con riduzione della pigmentazione basale, un infiltrato mononucleare perivascolare nella porzione superficiale del derma, fasci grossolani disorganizzati e ialinizzati di collagene, e la perdita variabile delle fibre elastiche nel derma."},"nl":{"name":"Confetti-achtige macula-atrofie","definition":"Een zeldzame, verworven stoornis van het elastische weefsel in de dermis met verminderde hoeveelheid elastisch weefsel, gekarakteriseerd door multipele, asymptomatische, goed begrensde, vlakke, gehypopigmenteerde, atrofische, maculaire huidletsels verspreid over het bovenste deel van de romp en de proximale delen van de bovenste ledematen. Histopathologisch onderzoek toont atrofische epidermis met verminderde basale pigmentatie, perivasculaire mononucleaire infiltratie in de bovenste dermis, gedesorganiseerde, gehyaliniseerde, ruwe collageenbundels, en variabel verlies van elastische vezels in de dermis."},"pt":{"name":"Atrofia macular confetti-like"},"pl":{"name":"Zanik plamki podobny do konfetti"},"cs":{"name":"Makulární atrofie podobná konfetám"},"uk":{"name":"Конфетті-подібна макулярна атрофія","definition":"A rare, acquired, dermis elastic tissue disorder with decreased elastic tissue characterized by multiple, asymptomatic, well demarcated, flat, hypopigmented atrophic macular skin lesions distributed over upper trunk and proximal upper limbs. Histopathological examination reveals atrophic epidermis with decreased basal pigmentation, perivascular mononuclear infiltration in the upper dermis, and disorganized, hyalinized, coarse collagen bundles, and variable loss of elastic fibers in the dermis."},"zh":{"name":"彩色纸屑样斑状萎缩","definition":"一种罕见的获得性真皮弹性组织疾病伴弹性组织减少，其特征是多发、无症状、界限清楚、扁平、色素缺乏的萎缩性黄斑皮肤病变，分布于上肢和上肢近端。组织病理学检查显示表皮萎缩，基底色素沉着减少，真皮上部血管周围单核细胞浸润，真皮中胶原纤维排列紊乱、透明化、粗糙，弹性纤维缺失。"},"tr":{"name":"Konfeti benzeri maküler atrofi"}},"xrefs":[{"source":"ICD-10","code":"L90.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE41.1","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0016376","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5687155","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":[],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":2.0,"area":"Worldwide","status":"Validated","source":"19878392[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_19878392[PMID]"}],"phenotypes":null}