{"orpha":90285,"name":"Lupus erythematosus panniculitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Lupus erythematosus profundus"],"definition":"A rare form of chronic cutaneous lupus erythematosus characterized by recurrent, indurated, erythematous plaques and subcutaneous nodules with normal overlying epidermis, occurring predominantly on the face, upper arms, trunk, buttocks, and thighs. The lesions can ulcerate and lead to scarring. Histological findings include lobular lymphocytic panniculitis, hyaline fat necrosis, mucin deposition, and calcification. The condition may be associated with discoid or systemic lupus erythematosus.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90285/","orphanet_url":"https://www.orpha.net/en/disease/detail/90285","names":{"fr":{"name":"Panniculite lupique","synonyms":["Lupus érythémateux profond"],"definition":"Forme rare de lupus érythémateux cutané chronique, caractérisée par des plaques érythémateuses et des nodules sous-cutanés indurés récurrents, avec un épiderme sus-jacent normal, survenant principalement sur le visage, la partie supérieure des bras, le tronc, les fesses et les cuisses. Les lésions peuvent s'ulcérer et entraîner des cicatrices. Les examens histologiques mettent en évidence une panniculite lymphocytaire lobulaire, une nécrose de la graisse hyaline, un dépôt de mucine et une calcification. Cette maladie peut être associée à un lupus érythémateux discoïde ou systémique."},"de":{"name":"Lupus erythematodes Pannikulitis","synonyms":["Lupus erythematodes profundus","Lupus-Pannikulitis"],"definition":"Eine seltene Form des chronischen kutanen Lupus erythematodes, die durch wiederkehrende, verhärtete, erythematöse Plaques und subkutane Knötchen mit normaler darüberliegender Epidermis gekennzeichnet ist und vorwiegend im Gesicht, an den Oberarmen, am Rumpf, Gesäß und an den Oberschenkeln auftritt. Die Läsionen können ulzerieren und zu Narbenbildung führen. Zu den histologischen Befunden gehören lobuläre lymphozytäre Pannikulitis, hyaline Fettnekrose, Muzinablagerungen und Kalzifizierung. Die Erkrankung kann mit diskoidalem oder systemischem Lupus erythematodes assoziiert sein."},"es":{"name":"Lupus eritematoso profundo","synonyms":["Paniculitis lúpica"],"definition":"Es una forma poco frecuente de lupus eritematoso cutáneo crónico caracterizado por placas eritematosas induradas, recurrentes y nódulos subcutáneos con una epidermis suprayacente normal, que aparecen predominantemente en la cara, la parte superior de los brazos, el tronco, las nalgas y los muslos. Las lesiones pueden ulcerarse, provocando cicatrices. Los hallazgos histológicos incluyen paniculitis lobulillar linfocítica, necrosis grasa hialina, depósitos de mucina y calcificación. El trastorno puede estar asociado a lupus eritematoso discoide o sistémico."},"it":{"name":"Panniculite da lupus eritematoso","synonyms":["Lupus eritematoso profondo"],"definition":"Si tratta di una forma rara di lupus eritematoso cutaneo cronico, caratterizzata da placche ricorrenti, indurite ed eritematose, e da noduli sottocutanei ricoperti da epidermide normale, localizzati per lo più sul viso, sulla porzione superiore del braccio, sul tronco, sui glutei e sulle cosce. Le lesioni possono ulcerarsi e lasciare cicatrici. L'esame istologico evidenzia pannicolite linfocitica lobulare, necrosi del grasso ialino, depositi di mucina e calcificazioni. La malattia può associarsi al lupus eritematoso discoide o sistemico."},"nl":{"name":"Lupus erythematosus panniculitis","synonyms":["Lupus erythematosus profundus"],"definition":"Een zeldzame vorm van chronische cutane lupus erythematosus, gekarakteriseerd door recidiverende, verharde, erythemateuze plaques en subcutane noduli met normale bovenliggende epidermis, vooral op aangezicht, bovenarm, romp, bil, en dij. De laesies kunnen verzweren en leiden tot littekenvorming. Histologische bevindingen zijn onder meer lobulaire lymfocytische panniculitis, hyaliene necrose van vetweefsel, depositie van mucine, en calcificatie. De aandoening kan geassocieerd zijn met discoïde of systemische lupus erythematosus."},"pt":{"name":"Lúpus eritematoso paniculite"},"pl":{"name":"Toczeń rumieniowaty głęboki","synonyms":["Lupus erythematosus profundus"]},"cs":{"name":"Lupus erythematodes panniculitis","synonyms":["Lupus erythematodes profundus"]},"uk":{"name":"Червоний вовчак панікуліт","synonyms":["Глибокий червоний вовчак"],"definition":"A rare form of chronic cutaneous lupus erythematosus characterized by recurrent, indurated, erythematous plaques and subcutaneous nodules with normal overlying epidermis, occurring predominantly on the face, upper arms, trunk, buttocks, and thighs. The lesions can ulcerate and lead to scarring. Histological findings include lobular lymphocytic panniculitis, hyaline fat necrosis, mucin deposition, and calcification. The condition may be associated with discoid or systemic lupus erythematosus."},"zh":{"name":"红斑狼疮性脂膜炎","synonyms":["深部红斑狼疮"]},"tr":{"name":"Lupus eritematozus panniküliti","synonyms":["Lupus eritematozus profundus"]}},"xrefs":[{"source":"ICD-10","code":"L93.2","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"EB51.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"84064653"},{"source":"MONDO","code":"0019561","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D015435","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0030327","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":163531,"name":"Chronic cutaneous lupus erythematosus","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood","Elderly","Infancy"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.07,"area":"United States","status":"Validated","source":"19289752[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.315,"area":"United States","status":"Validated","source":"9918242[PMID]_19289752[PMID]_30658703[PMID]_ORPHANET"}],"phenotypes":null}