{"orpha":228236,"name":"Linear focal elastosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Elastotic striae","Linear focal dermal elastosis"],"definition":"Linear focal elastosis is a rare, acquired, dermis elastic tissue disorder characterized by asymptomatic, palpable, hypertrophic or atrophic, yellowish or red, indurated, horizontal, striae-like linear plaques distributed symmetrically across the mid and lower back. No systemic involvement has been described. Skin biopsy reveals a focal increase in abnormal elastic tissue with abundant, wavy, fragmented and aggregated, basophilic elastic fibers in the reticular dermis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-228236/","orphanet_url":"https://www.orpha.net/en/disease/detail/228236","names":{"fr":{"name":"Elastose focale linéaire","definition":"Anomalie acquise et rare de l'élasticité du derme caractérisée par des plaques linéaires asymptomatiques, palpables, hypertrophiques ou atrophiques, jaunâtres ou rougeâtres, indurées, horizontales, en forme de stries, réparties de manière symétrique sur le milieu et le bas du dos. Aucune atteinte systémique n'a été décrite. La biopsie cutanée révèle des foyers de tissu élastique anormal avec d'abondants amas serpigineux et fragmentés de fibres élastiques basophiles dans le derme réticulaire."},"de":{"name":"Elastose, fokale lineare","synonyms":["Elastotische Striae","Lineare fokale dermale Elastose"]},"es":{"name":"Elastosis focal lineal","synonyms":["Elastosis dérmica focal lineal","Estrías elastóticas"],"definition":"Es un trastorno del tejido elástico de la dermis, adquirido y poco frecuente, caracterizado por unas placas lineales similares a estrías, asintomáticas, palpables, hipertróficas o atróficas, amarillentas o rojizas, endurecidas, horizontales, distribuidas simétricamente en la parte media y baja de la espalda. No se ha descrito ninguna afectación sistémica. La biopsia de piel revela un aumento focal de un tejido elástico anómalo con abundantes fibras elásticas basófilas, onduladas, fragmentadas y agrupadas en la dermis reticular."},"it":{"name":"Elastosi focale lineare","synonyms":["Strie elastotiche"],"definition":"L'elastosi focale lineare è una malattia rara acquisita del tessuto elastico del derma, caratterizzata da placche lineari sintomatiche, palpabili, ipertrofiche o atrofiche, giallastre o rosse, indurite, orizzontali, simili a striature, distribuite in modo simmetrico nella porzione centrale e inferiore della schiena. Non è presente un interessamento sistemico. La biopsia della cute evidenzia un aumento focale del tessuto elastico anomalo con abbondanti fibre elastiche basofile ondulate, frammentate ed aggregate nel derma reticolare."},"nl":{"name":"Lineaire focale elastose","synonyms":["Elastotische striae","Lineaire focale dermale elastose"],"definition":"Lineaire focale elastose is een zeldzame, verworven stoornis van elastische weefsel in de dermis, en wordt gekarakteriseerd door asymptomatische, tastbare, hyper- of atrofische, gelige of rode, verharde, horizontale, striemachtige lineaire plaques die symmetrisch verdeeld zijn over de midden- en onderrug. Er werd geen systemische betrokkenheid beschreven. Biopsie van de huid toont een focale toename van abnormaal elastisch weefsel met overvloedige, golvende, gefragmenteerde en geaggregeerde, basofiele elastische vezels in de reticulaire laag van de lederhuid."},"pt":{"name":"Elastose dérmica focal linear"},"pl":{"name":"Linijna elastoza ogniskowa skóry","synonyms":["Rozstępy elastotyczne"],"definition":"*Linijna ogniskowa elastoza skóry jest rzadkim, nabytym zaburzeniem tkanki sprężystej skóry właściwej, które charakteryzuje się bezobjawowymi, palpacyjnie wyczuwalnymi, przerostowymi lub zanikowymi, żółtawymi lub czerwonymi, stwardniałymi, poziomymi, linijnymi blaszkami przypominającymi prążki, rozmieszczonymi symetrycznie w środkowej i dolnej części pleców. Nie opisano objawów ogólnoustrojowych. W biopsji skóry widoczne są ogniska nieprawidłowej tkanki sprężystej z obfitymi, falistymi, pofragmentowanymi skupiskami bazofilnych włóknień sprężystych w warstwie siateczkowatej skóry właściwej.inf brsup"},"cs":{"name":"Lineární fokální dermální elastóza","synonyms":["Elastotické strie"]},"uk":{"name":"Лінійний вогнищевий еластоз","synonyms":["Еластичні смуги","Лінійний вогнищевий дермальний еластоз"],"definition":"Linear focal elastosis is a rare, acquired, dermis elastic tissue disorder characterized by asymptomatic, palpable, hypertrophic or atrophic, yellowish or red, indurated, horizontal, striae-like linear plaques distributed symmetrically across the mid and lower back. No systemic involvement has been described. Skin biopsy reveals a focal increase in abnormal elastic tissue with abundant, wavy, fragmented and aggregated, basophilic elastic fibers in the reticular dermis."},"zh":{"name":"线性局灶性弹性组织变性","synonyms":["线性局灶性真皮弹性组织变性"],"definition":"线状局灶性皮肤弹性组织变性是一种罕见的获得性真皮弹性组织疾病，其特征是无症状、可触及的、肥大或萎缩、黄或红、硬结的、水平、条纹状线状斑块，对称分布于背部中下部，没有全身性受累。皮肤活检显示网状真皮中有大量、波浪状、碎片状和集聚的嗜碱性弹性纤维，这些异常弹性组织局灶性增多。"},"tr":{"name":"Lineer fokal elastoz","synonyms":["Elastotik çizgiler","Doğrusal fokal dermal elastoz"]}},"xrefs":[{"source":"ICD-10","code":"L98.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE41.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0016438","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4751166","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":228224,"name":"Acquired dermis elastic tissue disorder with increased elastic tissue","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":30.0,"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}