{"orpha":79099,"name":"Interstitial granulomatous dermatitis with arthritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Ackerman dermatitis syndrome","Ackerman syndrome","IGDA"],"definition":"Interstitial granulomatous dermatitis with arthritis is a rare rheumatologic disease characterized by the occurrence of inflammatory arthritis in association with large, erythematous, symmetrical cutaneous lesions (ranging from typical, but infrequent, cord-like lesions on the flanks to more common violaceous plaques on the trunk and limbs) featuring a typical histologic infiltrate mainly constituted of histiocytes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-79099/","orphanet_url":"https://www.orpha.net/en/disease/detail/79099","names":{"fr":{"name":"Syndrome de dermatite granulomateuse interstitielle-arthrite","synonyms":["Dermatite d'Ackerman","Syndrome d'Ackerman"],"definition":"Maladie rhumatologique rare caractérisée par l'apparition d'une arthrite inflammatoire associée à des lésions cutanées érythémateuses étendues, disposées de manière symétrique (allant de lésions typiques, mais peu fréquentes, en forme de cordons sur les flancs à des plaques violacées plus fréquentes sur le tronc et les membres) présentant sur la plan histologique un infiltrat typique principalement constitué d'histiocytes."},"de":{"name":"Dermatitis, granulomatöse interstitielle mit Arthritis","synonyms":["Ackerman-Dermatitis-Syndrom","Ackerman-Syndrom","IGDA"]},"es":{"name":"Dermatitis granulomatosa intersticial con artritis","synonyms":["IGDA","Síndrome de Ackerman","Síndrome de dermatitis de Ackerman"],"definition":"Es una enfermedad reumatológica poco frecuente caracterizada por la aparición de artritis inflamatoria en asociación con lesiones cutáneas grandes, simétricas y eritematosas (que van desde lesiones típicas, aunque infrecuentes, de tipo cordón en flancos, hasta placas violáceas más comunes en tronco y extremidades) que presentan un infiltrado histológico típico constituido principalmente por histiocitos."},"it":{"name":"Dermatite granulomatosa interstiziale con artrite","synonyms":["Dermatite di Ackerman","IGDA"],"definition":"La dermatite granulomatosa interstiziale con artrite è una malattia reumatica rara, caratterizzata da artrite infiammatoria associata a lesioni cutanee eritematose simmetriche di grandi dimensioni, che vanno dalle lesioni tipiche a cordoncino sui fianchi (più rare) fino alle placche violacee sul tronco e sugli arti (più comuni), con un caratteristico infiltrato istologico costituito prevalentemente da istiociti."},"nl":{"name":"Interstitiële granulomateuze dermatitis met artritis","synonyms":["Dermatitissyndroom van Ackerman","IGDA","Syndroom van Ackerman"],"definition":"Interstitiële granulomateuze dermatitis met artritis is een zeldzame reumatologische ziekte die gekarakteriseerd wordt door het voorkomen van inflammatoire artritis in associatie met grote, erythemateuze, symmetrische huidletsels (gaande van typische, maar niet vaak voorkomende, strengachtige letsels op de flanken tot meer gangbare violette plaques op de romp en de ledematen) met een typisch histologisch infiltraat dat voornamelijk bestaat uit histiocyten."},"pt":{"name":"Dermatite granulomatosa intersticial com artrite","synonyms":["IGDA","Síndrome de dermatite de Ackerman"]},"pl":{"name":"Śródmiąższowe ziarniniakowe zapalenie skóry z zapaleniem stawów","synonyms":["IGDA","Zespół zapalenia skóry Ackermana"],"definition":"*Śródmiąższowe ziarniniakowe zapalenie skóry z zapaleniem stawów jest rzadką chorobą reumatologiczną, która charakteryzuje się występowaniem zapalenia stawów w połączeniu z dużymi, rumieniowatymi, symetrycznymi zmianami skórnymi (od typowych, ale rzadkich zmian przypominających sznurki na powierzchni bocznej ciała po częściej występujące fioletowe blaszki na tułowiu i kończynach) z typowym naciekiem, histologicznie składającym się głównie z histiocytów."},"cs":{"name":"Intersticiální granulomatózní dermatitida s artritidou","synonyms":["Ackermanuv dermatitický syndrom","IGDA","Ackermanův dermatitický syndrom"]},"uk":{"name":"Інтерстиціальний гранулематозний дерматит з артритом","synonyms":["Синдром акерманського дерматиту","Синдром Аккермана","IGDA"],"definition":"Interstitial granulomatous dermatitis with arthritis is a rare rheumatologic disease characterized by the occurrence of inflammatory arthritis in association with large, erythematous, symmetrical cutaneous lesions (ranging from typical, but infrequent, cord-like lesions on the flanks to more common violaceous plaques on the trunk and limbs) featuring a typical histologic infiltrate mainly constituted of histiocytes."},"zh":{"name":"间质肉芽肿性皮炎伴关节炎","synonyms":["Ackerman皮炎综合征"]},"tr":{"name":"Artrit ile seyreden interstisyel granülomatöz dermatit","synonyms":["IGDA","Ackerman sendromu","Ackerman dermatit sendromu"]}},"xrefs":[{"source":"ICD-10","code":"L30.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LD27.0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1946127088"},{"source":"MONDO","code":"0019199","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4751206","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":90077,"name":"Other acquired skin disease","classifications":[187]},{"orpha":182231,"name":"Rare rheumatologic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":53.0,"area":"Worldwide","status":"Validated","source":"22059717[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-09-23","source":"27274645[PMID]_26131871[PMID]","terms":[{"hpo":"HP:0010783","label":"Erythema","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001370","label":"Rheumatoid arthritis","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200034","label":"Papule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002923","label":"Rheumatoid factor positive","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Occasional (29-5%)","criteria":null}]}}