{"orpha":398127,"name":"Neonatal scleroderma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare secondary neonatal autoimmune disease characterized by neonatal-onset of erythematous skin lesions with a linear appearance that gradually become indurated and hyperpigmented and progressively present skin atrophy. Positive serum antibodies (in particular antinuclear antibodies and/or rheumatoid factor) may be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-398127/","orphanet_url":"https://www.orpha.net/en/disease/detail/398127","names":{"fr":{"name":"Sclérodermie néonatale","definition":"Maladie auto-immune néonatale secondaire rare caractérisée par l'apparition néonatale de lésions cutanées érythémateuses d'aspect linéaire qui deviennent progressivement indurées et hyperpigmentées et conduisent à une atrophie cutanée progressive. Des anticorps sériques positifs (en particulier des anticorps antinucléaires et/ou le facteur rhumatoïde) peuvent être associés."},"de":{"name":"Neonatale Sklerodermie"},"es":{"name":"Escleroderma neonatal","definition":"La esclerodermia neonatal es una enfermedad autoinmune neonatal secundaria y muy poco frecuente, caracterizada por la aparición neonatal de lesiones cutáneas eritematosas con un aspecto lineal que se endurece e hiperpigmenta gradualmente, presentando atrofia cutánea progresiva. Puede estar asociada la presencia de autoanticuerpos séricos (en particular, anticuerpos antinucleares y/o factor reumatoide)."},"it":{"name":"Sclerodermia neonatale","definition":"La sclerodermia neonatale è una malattia autoimmune secondaria molto rara, che esordisce nel periodo neonatale con lesioni cutanee eritematose dall'aspetto lineare, che gradualmente vanno incontro ad indurimento ed iperpigmentazione ed evolvono in una atrofia cutanea progressiva. Nel siero possono essere presenti anticorpi (in particolare gli anticorpi antinucleo e/o il fattore reumatoide)."},"nl":{"name":"Neonatale sclerodermie","definition":"Neonatale sclerodermie is een zeer zeldzame, secundaire, neonatale auto-immuunziekte, en wordt gekarakteriseerd door neonatale aanvang van erythemateuze huidletsels met een lineair uiterlijk die geleidelijk verharden en gehyperpigmenteerd worden, en progressief atrofie van de huid vertonen. Positieve antilichamen in het serum (voornamelijk antinucleaire antilichamen en/of reumafactor) kunnen geassocieerd zijn."},"pt":{"name":"Esclerodermia neonatal"},"pl":{"name":"Twardzina noworodków","definition":"*Twardzina noworodków jest bardzo rzadką, wtórną, noworodkową chorobą autoimmunologiczną, która charakteryzuje rumieniowatymi, linijnymi zmianami skórnymi o początku w okresie noworodkowym, które stopniowo ulegają stwardnieniu i przebarwieniu, prowadząc do postępującego zaniku skóry. W surowicy mogą pojawić się przeciwciała (w szczególności przeciwciała przeciwjądrowe i/lub czynnik reumatoidalny)."},"cs":{"name":"Neonatální sklerodermie"},"uk":{"name":"Неонатальна склеродермія","definition":"A rare secondary neonatal autoimmune disease characterized by neonatal-onset of erythematous skin lesions with a linear appearance that gradually become indurated and hyperpigmented and progressively present skin atrophy. Positive serum antibodies (in particular antinuclear antibodies and/or rheumatoid factor) may be associated."},"zh":{"name":"新生儿硬皮病","definition":"新生儿硬皮病是一种非常罕见的继发性新生儿自身免疫性疾病，以新生儿起病的红斑性皮肤损害为特征，呈线状，逐渐硬化和色素沉着，呈进行性皮肤萎缩。血清抗体阳性(特别是抗核抗体和/或类风湿因子)可能与本病相关。"},"tr":{"name":"Yenidoğan skleroderması"}},"xrefs":[{"source":"ICD-10","code":"P83.8","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"KC22.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018361","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4509425","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":398091,"name":"Secondary neonatal autoimmune disease","classifications":[196,231]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Neonatal"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":6.0,"area":"Worldwide","status":"Validated","source":"16887444[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_16887444[PMID]"}],"phenotypes":null}