{"orpha":178311,"name":"Isolated sternocostoclavicular hyperostosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Isolated SCCH"],"definition":"Isolated sternocostoclavicular hyperostosis is a rare rheumatologic disease characterized by predominantly bilateral, chronic, sterile inflammation and progressive sclerosis and hyperostosis of the sternocostoclavicular joint, with adjacent soft tissue ossification, in the absence of other joint involvement. It presents as recurrent episodes of pain, edema and/or erythema of the sternoclavicular region. Palmoplantar pustulosis may be additionally observed in some cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178311/","orphanet_url":"https://www.orpha.net/en/disease/detail/178311","names":{"fr":{"name":"Hyperostose sterno-costo-claviculaire isolée","definition":"Une maladie rhumatologique rare caractérisée par une inflammation stérile, chronique, principalement bilatérale, et par une sclérose et une hyperostose progressives de l'articulation sterno-costo-claviculaire, associées à une ossification du tissu mou adjacent, en l'absence d'autre atteinte articulaire. Elle se manifeste par des épisodes récurrents de douleur, d'oedème et/ou d'érythème de la région sterno-claviculaire. Une pustulose palmoplantaire peut aussi s'observer dans certains cas."},"de":{"name":"Hyperostose, isolierte sternokostoklavikuläre","synonyms":["SCCH, isolierte"],"definition":"Die isolierte sternokostoklavikuläre Hyperostose ist eine seltene rheumatologische Erkrankung, die durch eine überwiegend bilaterale, chronische, sterile Entzündung und fortschreitende Sklerose und Hyperostose des Sternokostoklavikulargelenks mit angrenzender Weichteilverknöcherung gekennzeichnet ist, ohne dass andere Gelenke betroffen sind. Die Erkrankung äußert sich durch rezidivierende Episoden von Schmerzen, Ödemen und/oder Erythemen in der Sternoklavikularregion. In einigen Fällen kann zusätzlich eine palmoplantare Pustulose beobachtet werden."},"es":{"name":"Hiperostosis esternocostoclavicular aislada","synonyms":["SCCH aislado"],"definition":"Es una enfermedad reumatológica poco frecuente caracterizada por inflamación estéril, crónica, predominantemente bilateral y esclerosis progresiva e hiperostosis de la articulación esternocostoclavicular, con osificación adyacente de los tejidos blandos, en ausencia de otra afectación articular. Se presenta con episodios recurrentes de dolor, edema y/o eritema de la región esternoclavicular. En algunos casos, también puede observarse pustulosis palmoplantar."},"it":{"name":"Iperostosi isolata sterno-costo-clavicolare","definition":"L'iperostosi isolata sterno-costo-clavicolare è una malattia reumatologica rara, caratterizzata da infiammazione sterile cronica, per lo più bilaterale, dell'articolazione sterno-costo-clavicolare, associata a sclerosi progressiva e iperostosi, con ossificazione del tessuto molle adiacente, senza interessamento di altre articolazioni. Esordisce con episodi ricorrenti di dolore, edema e/o eritema della regione sterno-clavicolare. Alcuni pazienti presentano pustolosi palmo-plantare."},"nl":{"name":"Geïsoleerde sternocostoclaviculaire hyperostose","synonyms":["Geïsoleerde SCCH"],"definition":"Geïsoleerde sternocostoclaviculaire hyperostose is een zeldzame reumatologische ziekte die gekarakteriseerd wordt door overwegend bilaterale, chronische, steriele inflammatie en progressieve sclerose en hyperostose van het sternocostoclaviculaire gewricht, met ossificatie van aangrenzende weke delen, en zonder betrokkenheid van andere gewrichten. Het presenteert zich met recurrente episodes van pijn, oedeem en/of erytheem in het sternoclaviculaire gebied. Bijkomend kan in sommige gevallen palmoplantaire pustulose waargenomen worden."},"pt":{"name":"Hiperostose esternocostoclavicular isolada"},"pl":{"name":"Izolowana hiperostoza mostkowo-żebrowo-obojczykowa","synonyms":["Isolated SCCH"],"definition":"*Izolowana hiperostoza mostkowo-żebrowo-obojczykowa jest rzadką chorobą reumatologiczną, która przeważnie charakteryzuje się obustronnym, przewlekłym, niebakteryjnym stanem zapalnym oraz postępującym stwardnieniem oraz hiperostozą stawu mostkowo-żebrowo-obojczykowego, z kostnieniem sąsiadujących tkanek miękkich, bez zajęcia innych stawów. Objawia się nawracającymi epizodami bólu, obrzęku i/lub zaczerwienienia okolicy mostkowo-obojczykowej. W niektórych przypadkach mogą wystąpić zmiany krostkowe na dłoniach i podeszwach."},"cs":{"name":"Izolovaná sternokostoklavikulární hyperostóza","synonyms":["Syndrom SAPHO"]},"uk":{"name":"Ізольований грудинно-реберно-ключичний гіперостоз","synonyms":["Ізольований SCCH"],"definition":"Isolated sternocostoclavicular hyperostosis is a rare rheumatologic disease characterized by predominantly bilateral, chronic, sterile inflammation and progressive sclerosis and hyperostosis of the sternocostoclavicular joint, with adjacent soft tissue ossification, in the absence of other joint involvement. It presents as recurrent episodes of pain, edema and/or erythema of the sternoclavicular region. Palmoplantar pustulosis may be additionally observed in some cases."},"zh":{"name":"孤立性胸肋锁骨肥厚症","synonyms":["孤立性SCCH"],"definition":"孤立性胸骨锁骨骨质增生症是一种罕见的风湿病，其主要表现为双侧慢性无菌性炎症、进行性硬化症和胸锁关节骨质增生，伴有邻近软组织骨化，不伴有其他关节受累。具体表现为反复发作的胸骨锁骨区疼痛、水肿和/或红斑，部分患者可出现掌跖脓疱病。"},"tr":{"name":"İzole sternokostoklaviküler hiperostoz","synonyms":["İzole SCCH"]}},"xrefs":[{"source":"ICD-10","code":"M85.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0044355","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707796","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182231,"name":"Rare rheumatologic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}