{"orpha":90159,"name":"Panniculitis-induced localized lipodystrophy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Panniculitis-induced localized lipodystrophy is a rare, acquired, localized lipodystrophy disorder characterized by eruption of tender, occasionally painful, erythematous nodules and plaques which enlarge radially and resolve into lipoatrophic lesions, often located in the upper and lower limbs. Histologically, lesions are characterized by lipophagic, lobular panniculitis and absence of vasculitis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90159/","orphanet_url":"https://www.orpha.net/en/disease/detail/90159","names":{"fr":{"name":"Lipodystrophie localisée induite par une panniculite","definition":"Lipodystrophie localisée acquise rare caractérisée par l'éruption de nodules et de plaques érythémateuses de consistance molle, parfois douloureuses, qui s'élargissent de manière radiale et se transforment en lésions lipoatrophiques, souvent localisées sur les membres supérieurs et inférieurs. Sur le plan histologique, les lésions sont caractérisées par une panniculite lobulaire lipophagique sans vascularite."},"de":{"name":"Lokale Lipodystrophie durch Pannikulitis"},"es":{"name":"Lipodistrofia localizada inducida por paniculitis","definition":"Es un trastorno lipodistrófico localizado, adquirido y poco frecuente, que se caracteriza por la erupción de placas y nódulos eritematosos blandos, en ocasiones dolorosos, que se agrandan radialmente originando unas lesiones lipoatróficas, localizadas típicamente en los miembros superiores e inferiores. Histológicamente, las lesiones se caracterizan por una paniculitis lobulillar lipofágica sin vasculitis."},"it":{"name":"Lipodistrofia localizzata indotta dalla panniculite","definition":"La lipodistrofia localizzata indotta dalla panniculite è una lipodistrofia localizzata acquisita rara, caratterizzata dalla comparsa di noduli eritematosi soffici, occasionalmente dolorosi, e di placche che si ingrandiscono radialmente e si risolvono lasciando lesioni lipoatrofiche, spesso localizzate negli arti superiori e inferiori. Dal punto di vista istologico, le lesioni sono caratterizzate da panniculite lobulare lipofagica, in assenza di vasculite."},"nl":{"name":"Panniculitis-geïnduceerde gelokaliseerde lipodystrofie","definition":"Panniculitis-geïnduceerde gelokaliseerde lipodystrofie is een zeldzame, verworven, gelokaliseerde lipodystrofie en wordt gekarakteriseerd door eruptie van gevoelige, soms pijnlijke, erythemateuze noduli en plaques die radiaal vergroten en verworden tot lipoatrofische letsels, vaak gesitueerd op de bovenste en onderste ledematen. Histologisch worden de letsels gekarakteriseerd door lipofagische, lobulaire panniculitis en afwezigheid van vasculitis."},"pt":{"name":"Paniculite e lipodistrofia localizada"},"pl":{"name":"Zapalenie tkanki podskórnej i lipodystrofia zlokalizowana"},"cs":{"name":"Panniculitida a lokalizovaná lipodystrofie"},"uk":{"name":"Локалізована ліподистрофія, спричинена панікулітом","definition":"Panniculitis-induced localized lipodystrophy is a rare, acquired, localized lipodystrophy disorder characterized by eruption of tender, occasionally painful, erythematous nodules and plaques which enlarge radially and resolve into lipoatrophic lesions, often located in the upper and lower limbs. Histologically, lesions are characterized by lipophagic, lobular panniculitis and absence of vasculitis."},"zh":{"name":"脂膜炎诱导局限性脂肪营养不良"},"tr":{"name":"Panikülite bağlı lokalize lipodistrofi"}},"xrefs":[{"source":"ICD-10","code":"E88.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EF01.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"493326096"},{"source":"MONDO","code":"0019555","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"19555","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190785","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79088,"name":"Localized lipodystrophy","classifications":[187,193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-04-19","source":"2613949[PMID]_3510652[PMID]_15554737[PMID]_18333902[PMID]_21865368[PMID]","terms":[{"hpo":"HP:0100578","label":"Lipoatrophy","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0003758","label":"Reduced subcutaneous adipose tissue","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010701","label":"Abnormal immunoglobulin level","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007485","label":"Absence of subcutaneous fat","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200036","label":"Skin nodule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003493","label":"Antinuclear antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200029","label":"Vasculitis in the skin","frequency":"Occasional (29-5%)","criteria":null}]}}