{"orpha":231031,"name":"Erythema palmare hereditarium","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Lane disease","Red palms disease"],"definition":"Erythema palmare hereditarium is a rare, benign, congenital genetic skin disorder characterized by permanent and asymptomatic erythema of the palmar and, less frequently, the solar surfaces. In most cases, it presents with sharply demarcated redness of the thenar and hypothenar eminences, as well as the palmar aspect of the phalanges, with scattered telangiectasia spots that do not cause any discomfort (pain, itching or burning) to the patient.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231031/","orphanet_url":"https://www.orpha.net/en/disease/detail/231031","names":{"fr":{"name":"Erythème palmaire héréditaire","synonyms":["Erythème palmoplantaire symétrique héréditaire","Maladie de Lane","Syndrome des paumes rouges"],"definition":"L'érythème palmaire héréditaire est une maladie génétique cutanée rare, congénitale et bénigne. Il se caractérise par un érythème permanent et asymptomatique des surfaces palmaires, et plus rarement, des plantes des pieds. Dans la plupart des cas, il se manifeste par une rougeur très délimitée des éminences thénar et hypothénar, ainsi que par l'aspect palmaire des phalanges, avec des taches télangiectasiques dispersées, n'entraînant pas d'inconfort chez le patient (douleur, démangeaison ou brûlure)."},"de":{"name":"Erythema palmare hereditarium","synonyms":["Hereditäres Erythem der Handinnenfläche","Lane-Krankheit","Syndrom der roten Palmae"],"definition":"Das Erythema palmare hereditarium ist eine seltene, gutartige, angeborene genetische Hauterkrankung, die durch ein dauerhaftes und asymptomatisches Erythem der palmaren und seltener der solaren Oberflächen gekennzeichnet ist. In den meisten Fällen zeigt sich eine scharf begrenzte Rötung der thenaren und hypothenaren Eminenzen sowie der palmaren Seite der Phalangen mit verstreuten Teleangiektasien, die dem Patienten keine Beschwerden (Schmerzen, Juckreiz oder Brennen) verursachen."},"es":{"name":"Eritema palmar hereditario","synonyms":["Enfermedad de Lane","Síndrome de las palmas rojas"],"definition":"El eritema palmar hereditario es un trastorno cutáneo genético congénito benigno y poco frecuente caracterizado por un eritema permanente y asintomático de las superficies palmares y, menos frecuentemente, de las plantares. En la mayoría de los casos, se presenta como un enrojecimiento muy bien delimitado de las eminencias tenares e hipotenares, así como de la cara palmar de las falanges, con máculas telangiectásicas dispersas que no causan ninguna sintomatología (dolor, picor o quemadura)."},"it":{"name":"Eritema palmare ereditario","synonyms":["Malattia dei palmi rossi","Malattia di Lane"],"definition":"L'eritema palmare ereditario è una malattia cutanea genetica rara, benigna e congenita. È caratterizzata dalla presenza di eritema asintomatico e permanente sulla superficie dei palmi e, più raramente, sulla superficie della pianta dei piedi. Nella maggior parte dei casi, esordisce con un rossore nettamente delimitato sulle eminenze tenar e ipotenar e sulla regione palmare delle falangi, con macchie telangectasiche che non comportano disturbi particolari (dolore, prurito o bruciore)."},"nl":{"name":"Erythema palmare hereditarium","synonyms":["Erfelijk erytheem van handpalm","Rode palmen-ziekte","Ziekte van Lane"],"definition":"Erythema palmaris hereditarium is een zeldzame, goedaardige, congenitale genetische huidziekte die wordt gekarakteriseerd door permanent en asymptomatisch erytheem aan de handpalmen en, minder frequent, de voetzolen. In de meeste gevallen uit de aandoening zich met scherp afgelijnde roodheid aan de duim- en pinkmuis, alsook aan de palmzijde van de vingers, met verspreide teleangiëctasieën die geen ongemak veroorzaken (pijn, jeuk of branderig gevoel) voor de patiënt."},"pt":{"name":"Eritema palmar hereditário"},"pl":{"name":"Dziedziczny rumień dłoniowy","synonyms":["Choroba Lane'a","Choroba czerwonych dłoni"]},"cs":{"name":"Erythema palmaris hereditarium","synonyms":["Laneova nemoc","Nemoc červených dlaní"]},"uk":{"name":"Спадкова еритема долонь","synonyms":["Хвороба Лейна","Хвороба червоних долонь"],"definition":"Erythema palmare hereditarium is a rare, benign, congenital genetic skin disorder characterized by permanent and asymptomatic erythema of the palmar and, less frequently, the solar surfaces. In most cases, it presents with sharply demarcated redness of the thenar and hypothenar eminences, as well as the palmar aspect of the phalanges, with scattered telangiectasia spots that do not cause any discomfort (pain, itching or burning) to the patient."},"zh":{"name":"遗传性掌红斑","synonyms":["红掌病"],"definition":"遗传性掌红斑是一种罕见的良性先天性皮肤遗传病，其特征是手掌和日光照射部位（不常见）的永久性无症状红斑。在大多数情况下，它表现为大鱼际、小鱼际以及指骨掌侧边界清晰的红色、分散的毛细血管扩张点，不会对患者造成任何不适（疼痛、瘙痒或灼热）。"},"tr":{"name":"Eritema palmare hereditarium","synonyms":["Lane hastalığı","Kırmızı avuç içi hastalığı"]}},"xrefs":[{"source":"OMIM","code":"133000","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"L98.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EM0Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0007570","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C565041","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1851502","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79385,"name":"Unclassified genetic skin disorder","classifications":[156,187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}