{"orpha":90158,"name":"Idiopathic localized lipodystrophy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Idiopathic localized lipodystrophy is a rare, acquired, localized lipodystrophy characterized by asymptomatic, well-demarcated, depressed, lipoatrophic lesions of variable size, with normal overlying skin without antecedent inflammation or a known identifiable cause (autoimmune disease, drug injection, injury, etc).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90158/","orphanet_url":"https://www.orpha.net/en/disease/detail/90158","names":{"fr":{"name":"Lipodystrophie localisée idiopathique","definition":"Une lipodystrophie localisée acquise rare, caractérisée par des lésions lipoatrophiques déprimées de taille variable, bien délimitées et asymptomatiques. La peau environnante est normale, sans antécédent d'inflammation ou de cause identifiable connue (maladie auto-immune, injection médicamenteuse, blessure, etc)."},"de":{"name":"Lipodystrophie, lokale, idiopathische","definition":"Die idiopathische lokalisierte Lipodystrophie ist eine seltene, erworbene, lokalisierte Lipodystrophie, die durch asymptomatische, gut abgegrenzte, niedergedrückte, lipoatrophische Läsionen unterschiedlicher Größe mit normaler darüberliegender Haut ohne vorangegangene Entzündung oder eine bekannte identifizierbare Ursache (Autoimmunerkrankung, Medikamenteninjektion, Verletzung usw.) gekennzeichnet ist."},"es":{"name":"Lipodistrofia idiopática localizada","definition":"Es un tipo de lipodistrofia localizada, adquirida y poco frecuente caracterizada por lesiones lipoatróficas asintomáticas, bien delimitadas, deprimidas, de tamaño variable, con una piel suprayacente de aspecto normal sin antecedentes previos de cambios inflamatorios o de una causa identificable (enfermedad autoinmune, inyección de fármacos, traumatismo, etc.) ."},"it":{"name":"Lipodistrofia localizzata idiopatica","definition":"La lipodistrofia localizzata idiopatica è una lipodistrofia rara acquisita, caratterizzata da lesioni lipoatrofiche depresse, asintomatiche, ben delimitate, di dimensioni variabili, ricoperte da cute normale, in assenza di una precedente infiammazione o di cause identificabili (malattie autoimmuni, iniezioni di farmaci, ferite, ecc.)"},"nl":{"name":"Idiopathische gelokaliseerde lipodystrofie","definition":"Idiopathische gelokaliseerde lipodystrofie is een zeldzame, verworven, gelokaliseerde lipodystrofie die gekarakteriseerd wordt door asymptomatische, goed begrensde, ingezonken, lipoatrofische letsels met een variabele omvang en met normale overliggende huid, zonder voorafgaande inflammatie of een gekende identificeerbare oorzaak (auto-immuunziekte, injectie van geneesmiddel/drug, verwonding, etc.)."},"pt":{"name":"Lipodistrofia localizada idiopática"},"pl":{"name":"Idiopatyczna zlokalizowana lipodystrofia","definition":"*Idiopatyczna zlokalizowana lipodystrofia jest rzadką, nabytą, zlokalizowaną lipodystrofią, która charakteryzuje się bezobjawowymi, dobrze odgraniczonymi, zagłębionymi, lipoatroficznymi zmianami o różnej wielkości, pokrytymi prawidłową skórą, i nie jest wywołana wcześniej występującym stanem zapalnym lub inną, możliwą do zidentyfikowania przyczyną (choroba autoimmunologiczna, iniekcja leku, uraz, itp.)."},"cs":{"name":"Idiopatická lokalizovaná lipodystrofie"},"uk":{"name":"Ідіопатична локалізована ліподистрофія","definition":"Idiopathic localized lipodystrophy is a rare, acquired, localized lipodystrophy characterized by asymptomatic, well-demarcated, depressed, lipoatrophic lesions of variable size, with normal overlying skin without antecedent inflammation or a known identifiable cause (autoimmune disease, drug injection, injury, etc)."},"zh":{"name":"特发性局限性脂质营养不良"},"tr":{"name":"İdiyopatik 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":"attributed","validated":true},{"source":"MONDO","code":"0019554","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0473566","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":["Adolescent","Adult","Childhood","Infancy"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":null,"value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-09-28","source":"8859277[PMID]_24346897[PMID]_18718199[PMID]_18280348[PMID]_17989899[PMID]_18718199[PMID]_8252774[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:0007485","label":"Absence of subcutaneous fat","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000953","label":"Hyperpigmentation of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001010","label":"Hypopigmentation of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012344","label":"Morphea","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0040189","label":"Scaling skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100324","label":"Scleroderma","frequency":"Very rare (<4-1%)","criteria":null}]}}