{"orpha":139402,"name":"Drug reaction with eosinophilia and systemic symptoms","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["DRESS syndrome","Drug rash with eosinophilia and systemic symptoms"],"definition":"A rare hypersensitivity reaction characterized by a generalized infiltrated skin rash with face edema, fever, enlarged lymph nodes, eosinophilia, lymphocytosis and more or less severe visceral involvement (e.g. hepatitis, nephritis, pneumonitis, myocarditis ect.) and, in some patients, reactivation of human herpes virus 6, Epstein-Barr virus and/or cytomegalovirus. Onset usually occurs 2-8 weeks after administration of the causal medication and is most frequently associated with anticonvulsants, antibacterial sulfonamides and allopurinol but many other medications have also been implicated. Histology is characterized by interface dermatitis, sometimes mixed with eczematous and an acute generalized exanthematous pustulosis-like pattern. Auto-immune sequelae may occur.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-139402/","orphanet_url":"https://www.orpha.net/en/disease/detail/139402","names":{"fr":{"name":"Réaction médicamenteuse avec éosinophilie et symptômes systémiques","synonyms":["Eruption cutanée avec éosinophilie et symptômes systémiques","Hypersensibilité médicamenteuse avec éosinophiles et manifestations systémiques","Syndrome DRESS"],"definition":"Réaction d'hypersensibilité rare caractérisée par une éruption cutanée infiltrée généralisée avec oedème du visage, fièvre, ganglions lymphatiques enflés, éosinophilie, lymphocytose et atteinte viscérale plus ou moins sévère (par exemple, hépatite, néphrite, pneumonie, myocardite, etc.) et, chez certains patients, par la réactivation du virus humain de l'herpès de type 6, du virus d'Epstein-Barr et/ou du cytomégalovirus. La maladie, qui se manifeste généralement entre deux et huit semaines après l'administration du médicament incriminé, est le plus souvent associée aux anticonvulsivants, aux sulfamides antibactériens et à l'allopurinol, mais de nombreux autres médicaments ont également été mis en cause. L'examen histologique est en faveur d'une dermatite d'interface, présentant parfois un aspect eczémateux et celui d'une pustulose exanthématique aiguë généralisée. Des séquelles auto-immunes sont possibles."},"de":{"name":"Arzneimittelreaktion mit Eosinophilie und systemischen Symptomen","synonyms":["DRESS-Syndrom","Hypersensitivitäts-Syndrom mit Eosinophilie und systemischen Symptomen"],"definition":"Eine seltene Überempfindlichkeitsreaktion, die durch einen generalisierten infiltrierten Hautausschlag mit Gesichtsödem, Fieber, vergrößerten Lymphknoten, Eosinophilie, Lymphozytose und einer mehr oder weniger schweren viszeralen Beteiligung (z. B. Hepatitis, Nephritis, Pneumonitis, Myokarditis usw.) sowie bei einigen Patienten durch die Reaktivierung des humanen Herpesvirus 6, des Epstein-Barr-Virus und/oder des Cytomegalovirus gekennzeichnet ist. Der Ausbruch tritt in der Regel 2-8 Wochen nach der Verabreichung des ursächlichen Medikaments auf und wird am häufigsten mit Antikonvulsiva, antibakteriellen Sulfonamiden und Allopurinol in Verbindung gebracht, aber auch viele andere Medikamente wurden in Betracht gezogen. Die Histologie ist gekennzeichnet durch eine Interface-Dermatitis, manchmal gemischt mit Ekzemen und einem akuten generalisierten exanthematischen pustuloseartigen Muster. Es können autoimmune Folgeerscheinungen auftreten."},"es":{"name":"Reacción a fármacos con eosinofilia y síntomas sistémicos","synonyms":["Síndrome DRESS","Erupción por fármacos con eosinofilia y síntomas sistémicos","Reacción a medicamentos con eosinofilia y síntomas sistémicos"],"definition":"Es una reacción de hipersensibilidad poco frecuente caracterizada por una erupción cutánea infiltrada generalizada con edema facial, fiebre, linfadenopatía, eosinofilia, linfocitosis y afectación visceral más o menos grave (como hepatitis, nefritis, neumonitis, miocarditis, etc.) y, en algunos pacientes, reactivación del virus del herpes humano 6, del virus de Epstein-Barr y/o de citomegalovirus. La aparición suele producirse entre 2 y 8 semanas después de la administración del medicamento causante y se asocia con mayor frecuencia a los anticonvulsivantes, las sulfonamidas antibacterianas y el alopurinol, pero también se han implicado muchos otros fármacos. Histológicamente se caracteriza por dermatitis de interfase, en ocasiones acompañada de un patrón eczematoso y pustulosis exantemática aguda generalizada. Pueden producirse secuelas de carácter autoinmune."},"it":{"name":"Reazione ai farmaci con eosinofilia e sintomi sistemici","synonyms":["Eruzione da farmaco con eosinofilia e sintomi sistemici","Sindrome DRESS"]},"nl":{"name":"Reactie op medicatie met eosinofilie en systemische symptomen","synonyms":["DRESS-syndroom","Medicatiegeïnduceerde huiduitslag met eosinofilie en systemische symptomen"],"definition":"Een zeldzame hypersensitieve reactie, gekenmerkt door een gegeneraliseerde geïnfiltreerde huiduitslag met oedeem van aangezicht, koorts, vergrote lymfeklieren, eosinofilie, lymfocytose en min of meer ernstige viscerale betrokkenheid (onder meer hepatitis, nefritis, pneumonitis, myocarditis), en in sommige patiënten reactivatie van humaan herpesvirus 6, Epstein-Barr virus en/of cytomegalovirus. Aanvang treedt meestal op vanaf 2-8 weken na toediening van de causale medicatie, en is het vaakst geassocieerd met anticonvulsiva, antibacteriële sulfonamiden en allopurinol, maar vele andere geneesmiddelen werden ook reeds geïmpliceerd. De histologie wordt gekenmerkt door grensvlakdermatitis, soms gemengd met een eczemateus en acuut gegeneraliseerd exanthemateus pustulose-achtig patroon. Auto-immune restverschijnselen kunnen optreden."},"pt":{"name":"Reação medicamentosa com eosinofilia e sintomas sistémicos","synonyms":["Exantema medicamentoso com eosinofilia e sintomas sistémicos","Síndrome DRESS"],"definition":"Reação de hipersensibilidade rara caracterizada por erupção cutânea com infiltração generalizada e edema facial, febre, aumento dos gânglios linfáticos, eosinofilia, linfocitose e envolvimento visceral com gravidade variável (por exemplo, hepatite, nefrite, pneumonite, miocardite, etc.) e, em alguns doentes, reativação do vírus do herpes humano 6, vírus Epstein-Barr e/ou citomegalovírus. O início geralmente ocorre 2 a 8 semanas após a administração do medicamento causal e é mais frequentemente associado a anticonvulsivantes, sulfonamidas antibacterianos e alopurinol, mas outros medicamentos também têm sido implicados. A histologia é caracterizada por dermatite de interface, por vezes combinada com eczema e um padrão agudo semelhante a pustulose exantemática generalizada. Podem ocorrer sequelas autoimunes."},"pl":{"name":"Reakcja polekowa z eozynofilią i objawami ogólnoustrojowymi","synonyms":["Polekowa eozynofilia z objawami ogólnymi","Zespół DRESS"],"definition":"Rzadka reakcja nadwrażliwości, która charakteryzuje się uogólnioną, naciekową wysypką skórną z obrzękiem twarzy, gorączką, powiększeniem węzłów chłonnych, eozynofilią, limfocytozą i mniej lub bardziej nasilonym zajęciem narządów trzewnych (np. zapaleniem wątroby, zapaleniem nerek, zapaleniem płuc, zapaleniem mięśnia sercowego itp.) oraz, u niektórych pacjentów, reaktywacją ludzkiego wirusa opryszczki 6, wirusa Epsteina-Barr i/lub wirusa cytomegalii. Początek choroby pojawia się zwykle 2-8 tygodni po podaniu leku sprawczego i jest najczęściej związany ze stosowaniem leków przeciwdrgawkowych, przeciwbakteryjnych sulfonamidów i allopurynolu, ale w patogenezę zaangażowanych może być także wiele innych leków. Obraz histologiczny charakteryzuje się powierzchniowym zapaleniem skóry, czasem w połączeniu z wypryskiem i ostrą uogólnioną osutką o wzorcu krostkowym. Mogą wystąpić powikłania autoimmunologiczne."},"cs":{"name":"Léky způsobená vyrážka s eozinofilií a systémovými symptomy","synonyms":["DRESS syndrom","Eozinofilní systémový syndrom způsobený reakcí na léčiva"]},"uk":{"name":"Медикаментозна реакція з еозинофілією та системними симптомами","synonyms":["DRESS-синдром","Медикаментозне висипання з еозинофілією та системними симптомами"],"definition":"A rare hypersensitivity reaction characterized by a generalized infiltrated skin rash with face edema, fever, enlarged lymph nodes, eosinophilia, lymphocytosis and more or less severe visceral involvement (e.g. hepatitis, nephritis, pneumonitis, myocarditis ect.) and, in some patients, reactivation of human herpes virus 6, Epstein-Barr virus and/or cytomegalovirus. Onset usually occurs 2-8 weeks after administration of the causal medication and is most frequently associated with anticonvulsants, antibacterial sulfonamides and allopurinol but many other medications have also been implicated. Histology is characterized by interface dermatitis, sometimes mixed with eczematous and an acute generalized exanthematous pustulosis-like pattern. Auto-immune sequelae may occur."},"zh":{"name":"伴嗜酸性粒细胞增多症和全身症状的药疹","synonyms":["药物反应嗜酸性粒细胞增多性系统性综合征"],"definition":"一种罕见的过敏反应，其特征是全身皮疹、发热、嗜酸粒细胞增多、淋巴细胞增多和内脏受累（肝炎、肾炎、肺炎、心包炎和心肌炎），并且在某些患者中出现了人类疱疹病毒6型的复活。"},"tr":{"name":"Eozinofili ve sistemik semptomlar ile seyreden ilaç döküntüsü","synonyms":["DRESS sendromu","İlaç reaksiyonu eozinofilik sistemik sendrom"]}},"xrefs":[{"source":"ICD-10","code":"T88.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EH65","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"516577496"},{"source":"MONDO","code":"0015340","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3541994","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13629","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073508","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":293815,"name":"Toxic dermatosis","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":"2016-06-01","source":null,"terms":[{"hpo":"HP:0100665","label":"Angioedema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001695","label":"Cardiac arrest","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001019","label":"Erythroderma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100326","label":"Immunologic hypersensitivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002383","label":"Infectious encephalitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012733","label":"Macule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100646","label":"Thyroiditis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006515","label":"Interstitial pneumonitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100827","label":"Lymphocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006554","label":"Acute hepatic failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030249","label":"Enanthema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012115","label":"Hepatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001880","label":"Increased total eosinophil count","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012819","label":"Myocarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000100","label":"Nephrotic syndrome","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001970","label":"Tubulointerstitial nephritis","frequency":"Occasional (29-5%)","criteria":null}]}}