{"orpha":293812,"name":"Fixed drug eruption","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare hypersensitivity reaction characterized by the appearance of erythematous or violaceous, round, sometimes painful, plaques that may or may not result in long-lasting pigmentation and which recur (usually at the same site) upon re-exposure to the causative medication. The severe form of the disease, generalized bullous fixed drug eruption, occurs typically in the elderly and may be life-threatening. Onset usually occurs 30 minutes to several hours after administration of the causal medication. Many medications, including paracetamol, have been implicated. The disease may rarely be induced by food. Histology is characterized by interface dermatitis or epidermal necrolysis in bullous forms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-293812/","orphanet_url":"https://www.orpha.net/en/disease/detail/293812","names":{"fr":{"name":"Erythème pigmenté fixe","definition":"Réaction d'hypersensibilité rare caractérisée par l'apparition de plaques rondes, violacées ou érythémateuses, parfois douloureuses, qui peuvent ou non entraîner une pigmentation de longue durée de la peau et qui récidivent (généralement au même endroit) après nouvelle exposition au médicament responsable. La forme grave de la maladie (érythème pigmenté fixe bulleux généralisé) survient généralement chez le sujet âgé et peut être létale. Elle se déclare généralement de 30 minutes à plusieurs heures après l'administration du médicament responsable. De nombreux médicaments, dont le paracétamol, ont été incriminés dans cette maladie. Dans de rares cas, la maladie peut être due à l'ingestion de certains aliments. L'examen histologique est caractérisé par une dermatite d'interface ou une nécrolyse épidermique de formes bulleuses."},"de":{"name":"Fixes Arzneimittelexanthem","definition":"Eine seltene Überempfindlichkeitsreaktion, die durch das Auftreten erythematöser oder violetter, runder, manchmal schmerzhafter Plaques gekennzeichnet ist, die zu einer lang anhaltenden Pigmentierung führen können oder auch nicht, und die bei erneuter Exposition gegenüber dem verursachenden Medikament erneut auftreten (in der Regel an derselben Stelle). Die schwere Form der Krankheit, die generalisierte bullöse fixe Arzneimittelreaktion, tritt typischerweise bei älteren Menschen auf und kann lebensbedrohlich sein. Der Ausbruch tritt in der Regel 30 Minuten bis mehrere Stunden nach Verabreichung des verursachenden Medikaments auf. Viele Medikamente, darunter auch Paracetamol, wurden in Betracht gezogen. Selten kann die Krankheit durch Nahrungsmittel ausgelöst werden. Die Histologie ist durch eine Oberflächendermatitis oder eine epidermale Nekrolyse bei bullösen Formen gekennzeichnet."},"es":{"name":"Erupción fija por fármacos","synonyms":["Erupción fija medicamentosa"],"definition":"Es una reacción de hipersensibilidad poco frecuente caracterizada por la aparición de placas eritematosas o violáceas, redondeadas, en ocasiones dolorosas, que pueden o no dar lugar a una pigmentación duradera y que reaparecen (normalmente en el mismo lugar) después de nuevas exposiciones al medicamento causante. La forma grave de la enfermedad, el eritema fijo ampolloso generalizado medicamentoso, se produce normalmente en personas de edad avanzada y puede poner en peligro la vida. La aparición suele producirse entre 30 minutos y varias horas después de la administración del fármaco causante. Se han implicado muchos medicamentos, incluido el paracetamol. Excepcionalmente, la enfermedad puede esrar inducida por los alimentos. Histológicamente se caracteriza por dermatitis de interfase o necrólisis epidérmica en las formas bullosas."},"it":{"name":"Eruzione fissa da farmaci","definition":"L'eruzione fissa da farmaci è una dermatosi tossica rara, caratterizzata dalla comparsa di un'eruzione indotta dai farmaci, che di solito si manifesta con piccole placche (di diametro inferiore a 8 cm) eritematose, arrotondate e talvolta dolorose, che esitano in una pigmentazione di lunga durata e che recidivano (in genere nella stessa zona) nel caso di una nuova assunzione del farmaco scatenante."},"nl":{"name":"Gefixeerde geneesmiddeleneruptie","definition":"Een zeldzame overgevoeligheidsreactie, gekenmerkt door het verschijnen van erythemateuze of violetkleurige, ronde, soms pijnlijke plaques die al dan niet resulteren in langdurige pigmentatie en die terugkeren (meestal op dezelfde plaats) bij hernieuwde blootstelling aan de causale medicatie. De ernstige vorm van de ziekte, gegeneraliseerde bulleuze 'fixed drug'-eruptie, treedt doorgaans op bij ouderen en kan levensbedreigend zijn. Aanvang treedt meestal op vanaf 30 minuten tot meerdere uren na toediening van de causale medicatie. Vele geneesmiddelen, waaronder paracetamol, werden reeds geïmpliceerd. In zeldzame gevallen kan de ziekte geïnduceerd worden door voedsel. De histologie wordt gekenmerkt door grensvlakdermatitis of epidermale necrolyse bij bulleuze vormen."},"pt":{"name":"Eritema pigmentado fixo","definition":"Reação de hipersensibilidade rara caracterizada pelo aparecimento de placas eritematosas ou violáceas, redondas, às vezes dolorosas, que podem ou não resultar em pigmentação duradoura e que são recorrentes (geralmente no mesmo local) após reexposição ao medicamento causador. A forma grave da doença, erupção bolhosa generalizada fixa por medicamento, ocorre tipicamente em idosos e pode ser fatal. O início geralmente ocorre 30 minutos a várias horas após a administração do medicamento causal. Muitos medicamentos, incluindo o paracetamol, foram implicados. A doença raramente pode ser induzida por alimentos. A histologia é caracterizada por dermatite de interface ou necrólise epidérmica nas formas bolhosas."},"pl":{"name":"Rumień barwnikowy trwały","definition":"Rzadka reakcja nadwrażliwości, która charakteryzuje się występowaniem rumieniowych lub fioletowo zabarwionych, okrągłych, czasem bolesnych blaszek, które mogą, ale nie muszą, powodować długotrwałą pigmentację i które nawracają (zwykle w tym samym miejscu) po ponownym podaniu leku wywołującego chorobę. Ciężka postać choroby, uogólniona pęcherzowa wysypka polekowa, występuje zwykle u osób starszych i może zagrażać życiu. Objawy pojawiają się zwykle od 30 minut do kilku godzin po podaniu leku sprawczego. W patogenezie choroby znaczenie może mieć wiele leków, w tym paracetamol. Rzadko choroba może być wywołana pokarmem. W obrazie histologicznym obserwuje się powierzchniowe zapalenie skóry lub martwica naskórka w postaci pęcherzowej."},"cs":{"name":"Fixovaný pigmentovaný erytém"},"uk":{"name":"Фіксований висип, викликаний ліками","definition":"A rare hypersensitivity reaction characterized by the appearance of erythematous or violaceous, round, sometimes painful, plaques that may or may not result in long-lasting pigmentation and which recur (usually at the same site) upon re-exposure to the causative medication. The severe form of the disease, generalized bullous fixed drug eruption, occurs typically in the elderly and may be life-threatening. Onset usually occurs 30 minutes to several hours after administration of the causal medication. Many medications, including paracetamol, have been implicated. The disease may rarely be induced by food. Histology is characterized by interface dermatitis or epidermal necrolysis in bullous forms."},"zh":{"name":"固定性药疹","definition":"固定性药疹是一种罕见的毒性皮肤病，其特征是出现小的圆形红斑样药物性皮疹（直径小于8厘米），有时疼痛，导致长期色素沉着，再次接触致病性药物时复发（常在同一部位）。"},"tr":{"name":"Sabit ilaç püskürmesi"}},"xrefs":[{"source":"ICD-10","code":"L25.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EH66","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"20014644"},{"source":"MONDO","code":"0017395","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"17395","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0221242","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10016740","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":"Validated","source":"ORPHANET_26548517[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-05-04","source":"32002848[PMID]_29469697[PMID]_31006486[PMID]","terms":[{"hpo":"HP:0020172","label":"Adverse drug response","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0410323","label":"Drug allergy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025474","label":"Erythematous plaque","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003341","label":"Junctional split","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011356","label":"Regional abnormality of skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000953","label":"Hyperpigmentation of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032156","label":"Skin detachment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010280","label":"Stomatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025143","label":"Chills","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0007473","label":"Crusting erythematous dermatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011354","label":"Generalized abnormality of skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0032565","label":"Vaginal mucosal ulceration","frequency":"Very rare (<4-1%)","criteria":null}]}}