{"orpha":231111,"name":"Drug-induced lupus erythematosus","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["DILE"],"definition":"A rare, systemic disease with skin involvement characterized by the onset of idiopathic lupus erythematosus-like signs and symptoms resulting from continuous drug intake (>1 month), which resolve when treatment is discontinued, in persons with no history of autoimmune disease. Manifestations are variable and may be systemic (e.g. arthralgia, myalgia, fever, fatigue, serositis, pleuritis, pericarditis), subacute cutaneous (incl. photosensitive, non-scarring, annular, polycyclic or papulosquamous lesions, malar erythema, vasculitis, bullous lesions, erythema multiforme-like changes), and/or chronic cutaneous (typically discoid lesions in sun-exposed areas). Procainamide and hydralazine are the drugs most frequently implicated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231111/","orphanet_url":"https://www.orpha.net/en/disease/detail/231111","names":{"fr":{"name":"Lupus érythémateux induit par les médicaments","synonyms":["DILE"],"definition":"Maladie systémique rare avec atteinte cutanée, caractérisée par l'apparition de signes et symptômes de type lupus érythémateux idiopathique après une prise continue de médicaments (> 1 mois) chez des personnes sans antécédent de maladie auto-immune, qui se résout avec l'arrêt du traitement. Les manifestations, inconstantes, peuvent être systémiques (arthralgie, myalgie, fièvre, fatigue, sérite, pleurésie, péricardite), cutanées subaiguës (dont lésions photosensibles, non cicatricielles, annulaires, polycycliques ou papulosquameuses, érythème malaire, vascularite, lésions bulleuses, lésions de type érythème polymorphe) et/ou cutanées chroniques (généralement lésions discoïdes sur les zones exposées au soleil). La procaïnamide et l'hydralazine sont les médicaments les plus fréquemment incriminés."},"de":{"name":"Lupus erythematodes, Medikamenteninduzierter","synonyms":["DILE"],"definition":"Eine seltene, systemische Erkrankung mit Hautbeteiligung, die durch das Auftreten von idiopathischem Lupus erythematodes-ähnlichen Anzeichen und Symptomen nach kontinuierlicher Medikamenteneinnahme (>1 Monat) gekennzeichnet ist, die nach Absetzen der Behandlung bei Personen ohne Autoimmunerkrankung in der Vorgeschichte wieder verschwinden. Die Manifestationen sind variabel und können systemisch (z. B. Arthralgie, Myalgie, Fieber, Müdigkeit, Serositis, Pleuritis, Perikarditis), subakut kutan (u. a. lichtempfindliche, nicht vernarbende, ringförmige, polyzyklische oder papulosquamöse Läsionen, malares Erythem, Vaskulitis, bullöse Läsionen, erythem multiforme-ähnliche Veränderungen) und/oder chronisch kutan (typischerweise diskoide Läsionen in sonnenexponierten Bereichen) sein. Procainamid und Hydrazin sind die am häufigsten eingesetzten Medikamente."},"es":{"name":"Lupus eritematoso inducido por fármacos","synonyms":["DILE","Lupus eritematoso inducido por medicamentos"],"definition":"Es una enfermedad sistémica de afectación cutánea poco frecuente caracterizada por la aparición de hallazgos y síntomas similares al lupus eritematoso idiopático resultantes de la ingesta continuada de medicamentos (>1 mes), que remiten al suspender el tratamiento en individuos sin antecedentes de enfermedad autoinmune. Las manifestaciones son variables y pueden ser sistémicas (p. ej. artralgia, mialgia, fiebre, fatiga, serositis, pleuritis, pericarditis), cutáneas subagudas (incluidas las lesiones fotosensibles, sin cicatrices, anulares, policíclicas o papuloescamosas, eritema malar, vasculitis, lesiones bullosas, eritema de tipo multiforme) y/o cutáneas crónicas (por lo general, lesiones discoides en áreas expuestas al sol). Los fármacos implicados con mayor frecuencia son la procainamida y la hidrazalina."},"it":{"name":"Lupus eritematoso indotto dai farmaci","synonyms":["DILE"],"definition":"È una malattia sistemica rara con coinvolgimento cutaneo, caratterizzata da segni clinici che ricordano il lupus eritematoso idiopatico, secondari all'assunzione continuativa (oltre un mese) di farmaci, che si risolvono quando il trattamento viene sospeso, in pazienti con anamnesi negativa per malattie autoimmuni. I segni clinici sono variabili, e possono essere sistemici (artralgia, mialgia, febbre, affaticamento, sierosite, pleurite, pericardite), cutanei subacuti (lesioni fotosensibili non cicatriziali anulari policicliche o papulosquamose, eritema malare, vasculite, lesioni bollose, alterazioni simil-eritema multiforme), e/o cutanei cronici (di solito lesioni discoidi nelle zone esposte al sole). I farmaci più indicati sono la procainamide e l'idralazina."},"nl":{"name":"Medicatiegeïnduceerde lupus erythematosus","synonyms":["DILE"],"definition":"Een zeldzame, systemische ziekte met betrokkenheid van de huid en gekarakteriseerd door aanvang van idiopathische lupus erythematosus-achtige tekenen en symptomen die veroorzaakt worden door aanhoudende inname van geneesmiddelen (>1 maand) en die verdwijnen wanneer de behandeling wordt stopgezet, bij personen zonder enige voorgeschiedenis van auto-immuunziekten. Manifestaties zijn variabel en mogelijk systemisch (e.g. artralgie, myalgie, koorts, vermoeidheid, serositis, pleuritis, pericarditis), subacuut cutaan (inclusief lichtgevoelige, niet-littekenvormende, ringvormige, polycyclische of papulosquameuze laesies, erytheem aan de jukbeenderen, vasculitis, bulleuze laesies, erythema multiforme-achtige veranderingen), en/of chronisch cutaan (doorgaans discoïde laesies in gebieden blootgesteld aan de zon). Procaïnamide en hydrazaline zijn de geneesmiddelen die het vaakst betrokken zijn."},"pt":{"name":"Lupus eritmatoso induzido por fármacos"},"pl":{"name":"Toczeń rumieniowaty indukowany lekami","synonyms":["DILE"]},"cs":{"name":"Léky navozený systémový lupus erythematodes","synonyms":["DILE"]},"uk":{"name":"Медикаментозний червоний вовчак","synonyms":["DILE"],"definition":"A rare, systemic disease with skin involvement characterized by the onset of idiopathic lupus erythematosus-like signs and symptoms resulting from continuous drug intake (>1 month), which resolve when treatment is discontinued, in persons with no history of autoimmune disease. Manifestations are variable and may be systemic (e.g. arthralgia, myalgia, fever, fatigue, serositis, pleuritis, pericarditis), subacute cutaneous (incl. photosensitive, non-scarring, annular, polycyclic or papulosquamous lesions, malar erythema, vasculitis, bullous lesions, erythema multiforme-like changes), and/or chronic cutaneous (typically discoid lesions in sun-exposed areas). Procainamide and hydralazine are the drugs most frequently implicated."},"zh":{"name":"药物诱发红斑狼疮","synonyms":["DILE"],"definition":"一种罕见的全身性皮肤病，其特征是在无自身免疫性疾病史的患者中，由于持续服用药物（ inf 1个月）而出现特发性红斑狼疮样体征和症状，当停止治疗时症状消失。临床表现多种多样，可能是全身性的（如关节痛、肌痛、发热、疲劳、浆膜炎、胸膜炎、心包炎）、亚急性皮肤病（包括光敏性、非瘢痕性、环状、多环或丘疹鳞状病变、颧颊部红斑、血管炎、大疱性病变、多形性红斑样改变），和/或慢性皮肤病（通常是阳光照射区域的盘状损害）。普鲁卡因胺和肼屈嗪是最常涉及的药物。"},"tr":{"name":"İlaca bağlı lupus eritematoz","synonyms":["DILE"]}},"xrefs":[{"source":"ICD-10","code":"M32.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"4A40.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1239818910"},{"source":"MONDO","code":"0016474","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0263591","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10013706","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182222,"name":"Rare systemic disease","classifications":[196]},{"orpha":290836,"name":"Systemic disease with skin involvement","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-07-03","source":"26420637[PMID]_26710505[PMID]","terms":[{"hpo":"HP:0001903","label":"Anemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003493","label":"Antinuclear antibody positivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030057","label":"Autoimmune antibody positivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005421","label":"Decreased circulating complement C3 concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0045042","label":"Decreased circulating complement C4 concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003236","label":"Elevated circulating creatine kinase 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:0000790","label":"Hematuria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003138","label":"Increased blood urea nitrogen","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025343","label":"Lupus anticoagulant","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025300","label":"Malar rash","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001698","label":"Pericardial effusion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001701","label":"Pericarditis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000967","label":"Petechiae","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005184","label":"Prolonged QTc interval","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0045073","label":"Serositis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025142","label":"Constitutional symptom","frequency":"Occasional (29-5%)","criteria":null}]}}