{"orpha":306553,"name":"Myospherulosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Spherulocytosis","Subcutaneous spherulocystic disease"],"definition":"A rare maxillo-facial surgical disease characterized by an inflammatory, granulomatous lesion, most commonly of iatrogenic origin due to interaction of extravasated erythrocytes with exogenous lipids, in particular petrolatum-based antibiotic ointment used after surgical procedures. Most frequent locations are the paranasal sinuses and jaws, although the lesion can occur in any part of the body. It is typically found incidentally as an asymptomatic soft tissue swelling.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-306553/","orphanet_url":"https://www.orpha.net/en/disease/detail/306553","names":{"fr":{"name":"Myosphérulose","synonyms":["Sphérulocytose"],"definition":"Maladie rare en chirurgie maxillo-faciale caractérisée par une lésion granulomateuse inflammatoire, le plus souvent d'origine iatrogène. Elle est due à l'interaction des érythrocytes extravasés avec des lipides exogènes, présents notamment dans une pommade antibiotique à base de pétrolatum appliquée à la suite des interventions chirurgicales. Bien que la lésion puisse toucher n'importe quelle partie du corps, on l'observe le plus souvent au niveau des sinus paranasaux et des mâchoires. Elle se présente généralement de manière fortuite sous la forme d'un gonflement asymptomatique des tissus mous."},"de":{"name":"Myospherulose","synonyms":["Spherulozytose"],"definition":"Eine seltene Erkrankung aus dem Fachbereich der Mund-, Kiefer- und Gesichtschirurgie, die durch eine entzündliche, granulomatöse Läsion gekennzeichnet ist, die in den meisten Fällen iatrogenen Ursprungs ist und auf die Interaktion von extravasierten Erythrozyten mit exogenen Lipiden, insbesondere mit antibiotischen Salben auf Petrolatumbasis, die nach chirurgischen Eingriffen verwendet werden, zurückzuführen ist. Die häufigsten Lokalisationen sind die Nasennebenhöhlen und der Kiefer, obwohl die Läsion an jeder Stelle des Körpers auftreten kann. Sie wird meist zufällig als asymptomatische Weichteilschwellung entdeckt."},"es":{"name":"Mioesferulosis","synonyms":["Enfermedad esferuloquística subcutánea","Esferulocitosis"],"definition":"Es una patología quirúrgica maxilofacial poco frecuente caracterizada por una lesión inflamatoria granulomatosa, más comúnmente de origen iatrogénico debido a la interacción de eritrocitos extravasados con lípidos exógenos, en particular por el uso de pomadas que contienen vaselina en la profilaxis antibiótica después de un procedimiento quirúrgico. Las localizaciones más frecuentes son los senos paranasales y la mandíbula, aunque la lesión puede ocurrir en cualquier localización anatómica. Por lo general, se encuentra de manera incidental como una inflamación asintomática en partes blandas."},"it":{"name":"Miosferulosi","synonyms":["Sferulocitosi"],"definition":"Si tratta di una malattia di interesse maxillofacciale, caratterizzata da una lesione infiammatoria granulomatosa, in genere di origine iatrogena, dovuta all'interazione tra gli eritrociti usciti dai vasi e i lipidi esogeni, in particolare quelli presenti nella pomata antibiotica a base di petrolato utilizzata dopo gli interventi chirurgici. Le localizzazioni più comuni sono i seni paranasali e le mascelle, ma la lesione può insorgere in qualsiasi parte del corpo. Di solito viene diagnosticata casualmente in presenza di un gonfiore asintomatico dei tessuti molli."},"nl":{"name":"Myosferulose","synonyms":["Sferulocytose","Subcutane sferulocystische ziekte"],"definition":"Een zeldzame maxillofaciale chirurgische aandoening, gekarakteriseerd door een inflammatoire, granulomateuze laesie, doorgaans van iatrogene oorsprong ten gevolge van interactie tussen extravasaat van erytrocyten en exogene lipiden, vooral antibiotische petrolatumzalf die gebruikt wordt na chirurgische ingrepen. De meest frequente locaties zijn paranasale sinussen en kaken, maar de laesie kan overal op het lichaam voorkomen . Doorgaans wordt het toevallig aangetroffen als een asymptomatisch zwelling van weke delen."},"pt":{"name":"Miosferulose"},"pl":{"name":"Sferulozytoza mięśniowa","synonyms":["Sferulozytoza"]},"cs":{"name":"Myosferulóza","synonyms":["Sferulocytóza"]},"uk":{"name":"Міосферульоз","synonyms":["Сфероцитоз","Підшкірна сферолоцитарна хвороба"],"definition":"A rare maxillo-facial surgical disease characterized by an inflammatory, granulomatous lesion, most commonly of iatrogenic origin due to interaction of extravasated erythrocytes with exogenous lipids, in particular petrolatum-based antibiotic ointment used after surgical procedures. Most frequent locations are the paranasal sinuses and jaws, although the lesion can occur in any part of the body. It is typically found incidentally as an asymptomatic soft tissue swelling."},"zh":{"name":"肌小球体病","synonyms":["皮下球样囊肿"],"definition":"一种罕见的颌面部外科疾病，特征为炎性肉芽肿性病变，常为医源性，因红细胞外渗和外源性脂质（特别是术后使用凡士林基质的抗生素软膏）相互作用所致。尽管病变可以发生在身体的任何部位，但副鼻窦和颌骨最为常见。常为偶然发现并认为是无症状软组织肿胀。"},"tr":{"name":"Miyosferüloz","synonyms":["Sferositoz","Deri altı sferülokistik hastalık"]}},"xrefs":[{"source":"ICD-10","code":"T88.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017628","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0027123","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68329,"name":"Rare maxillo-facial surgical disease"},"parents":[{"orpha":165711,"name":"Rare abdominal surgical disease","classifications":[182]},{"orpha":68329,"name":"Rare maxillo-facial surgical disease","classifications":[209]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}