{"orpha":293173,"name":"Acute generalized exanthematous pustulosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["AGEP","Pustular drug eruption","Toxic pustuloderma"],"definition":"A rare hypersensitivity reaction characterized by the rapid development of numerous, nonfollicular, sterile, pinhead-sized pustules on an erythematous base, predominantly occurring on the trunk, intertriginous and flexural areas, with rare, mostly oral, mucosal involvement. Fever, peripheral blood leukocytosis, and mild eosinophilia are accompanying features. Systemic involvement, with hepatic, renal or pulmonary dysfunction, occasionally occurs. Onset usually occurs 1-12 days after administration of the causal medication and is most frequently associated with beta&#8208;lactam antibiotics, macrolides (including pristinamycin and clindamycin), diltiazem, terbinafine, (hydroxy&#8208;)chloroquine but many other medications have also been implicated. Histology reveals spongiform, subcorneal and/or intraepidermal, pustules but this pattern is not specific (same in pustular psoriasis).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-293173/","orphanet_url":"https://www.orpha.net/en/disease/detail/293173","names":{"fr":{"name":"Pustulose exanthématique aiguë généralisée","synonyms":["AGEP","PEAG"],"definition":"Réaction d'hypersensibilité rare caractérisée par le développement rapide de nombreuses pustules non folliculaires, stériles, de la taille d'une tête d'épingle sur une lésion érythémateuse, siégeant principalement sur le tronc, les zones intertrigineuses et de flexion, avec de rares atteintes des muqueuses (généralement buccales). Une fièvre, une leucocytose sanguine périphérique et une légère éosinophilie sont associées. Une atteinte systémique, avec dysfonctionnement hépatique, rénal ou pulmonaire, est parfois observée. La maladie se déclare généralement 1 à 12 jours après l'administration du médicament incriminé et est le plus souvent associée à la prise d'antibiotiques de type bêtalactamines et macrolides (dont la pristinamycine et la clindamycine), ainsi qu'à la prise de diltiazem, de terbinafine et d'(hydroxy-)chloroquine. De nombreux autres médicaments ont également été incriminés. L'examen histologique révèle des pustules spongiformes, sous-cornéennes et/ou intra-épidermiques, mais cet aspect n'est pas spécifique de la maladie puisqu'on le retrouve aussi dans le psoriasis pustuleux."},"de":{"name":"Akute generalisierte exanthematische Pustulose","synonyms":["AGEP","Arzneimittelexanthem, pustulöses","Pustuloderm, toxisches"],"definition":"Eine seltene Hypersensitivitätsreaktion, die durch die rasche Entwicklung zahlreicher, nichtfollikulärer, steriler, stecknadelkopfgroßer Pusteln auf erythematösem Grund gekennzeichnet ist und vorwiegend am Rumpf, im intertriginösen Bereich und an den Beugen auftritt, mit seltener, meist oraler, Schleimhautbeteiligung. Fieber, Leukozytose im peripheren Blut und leichte Eosinophilie sind Begleitmerkmale. Gelegentlich kommt es zu einer systemischen Beteiligung mit Leber-, Nieren- oder Lungenfunktionsstörung. Der Ausbruch tritt in der Regel 1-12 Tage nach Verabreichung des ursächlichen Medikaments auf und wird am häufigsten mit Beta-Lactam-Antibiotika, Makroliden (einschließlich Pristinamycin und Clindamycin), Diltiazem, Terbinafin und (Hydroxy-)Chloroquin in Verbindung gebracht, aber auch viele andere Medikamente wurden in Betracht gezogen. Die Histologie zeigt spongiforme, subkorneale und/oder intraepidermale Pusteln, aber dieses Muster ist nicht spezifisch (wie bei der pustulösen Psoriasis)."},"es":{"name":"Pustulosis exantemática generalizada aguda","synonyms":["AGEP","Erupción pustulosa por fármacos","Pustuloderma tóxico"],"definition":"Es una reacción de hipersensibilidad poco frecuente caracterizada por el rápido desarrollo de numerosas pústulas no foliculares, estériles, del tamaño de una cabeza de alfiler, sobre una base eritematosa, que se originan predominantemente en el tronco y en las zonas intertriginosas y de flexión, con una rara afectación de la mucosa, principalmente oral. La fiebre, la leucocitosis en sangre periférica y la eosinofilia leve son características que acompañan a la enfermedad. Ocasionalmente se produce una afectación sistémica, con disfunción hepática, renal o pulmonar. El inicio suele producirse entre 1 y 12 días después de la administración del medicamento causante y se asocia con mayor frecuencia a los antibióticos beta&#8208;lactámicos, los macrólidos (incluyendo la pristinamicina y la clindamicina), el diltiazem, la terbinafina, la (hidroxi&#8208;)cloroquina, pero también se han implicado muchos otros fármacos. La histología revela pústulas espongiformes, subcorneales y/o intraepidérmicas, pero este patrón no es específico (al igual que en la psoriasis pustulosa)."},"it":{"name":"Pustolosi esantematosa generalizzata acuta","synonyms":["AGEP","Eruzione pustolosa da farmaci","Pustoloderma tossico"],"definition":"È una dermatosi tossica rara, caratterizzata dalla rapida comparsa di numerose pustole non follicolari sterili, delle dimensioni di una testa di spillo, su base edematosa ed eritematosa, localizzate prevalentemente sul tronco, sulle zone intertriginose e flessorie, con raro coinvolgimento delle mucose (per lo più quella orale). Si associa a febbre ad esordio acuto (>38°C), leucocitosi nel sangue periferico e lieve eosinofilia. Occasionalmente i pazienti presentano un quadro sistemico, con disfunzione epatica, renale o polmonare. Gli esami istologici evidenziano caratteristiche pustole spongiformi, subcorneali e/o intraepidermiche, edema marcato del derma papillare, infiltrato significativo infiammatorio perivascolare ricco in neutrofili, ed eosinofili intrapustolari o intradermici."},"nl":{"name":"Acute gegeneraliseerde exanthemateuze pustulose","synonyms":["AGEP","Pustuleuze eruptie door medicatie","Toxische pustulodermie"],"definition":"Een zeldzame overgevoeligheidsreactie, gekenmerkt door snelle ontwikkeling van talrijke, niet-folliculaire, steriele, speldenknop-vormige pustels op een erythemateuze basis, voornamelijk op romp, intertrigineuze delen en flexiegebieden, met zeldzame, meestal orale, betrokkenheid van slijmvlies. Koorts, leukocytose in perifeer bloed, en milde eosinofilie zijn bijbehorende kenmerken. Systemische betrokkenheid, met disfunctie van lever, nier of long, komt occasioneel voor. Aanvang treedt meestal op vanaf 1-12 dagen na toediening van de causale medicatie, en is het vaakst geassocieerd met bètalactam-antibiotica, macroliden (waaronder pristinamycine en clindamycine), diltiazem, terbinafine, en (hydroxy&#8208;)chloroquine, maar vele andere geneesmiddelen werden ook reeds geïmpliceerd. De histologie wordt gekenmerkt door spongiforme, subcorneale en/of intra-epidermale pustels, maar dit patroon is niet specifiek (identiek bij pustuleuze psoriasis)."},"pt":{"name":"Pustulose exantemática generalizada aguda","synonyms":["Erupção pustular por fármaco","PGEA","Pustulodermia tóxica"],"definition":"Reação de hipersensibilidade rara caracterizada pelo rápido desenvolvimento de numerosas pústulas não foliculares, estéreis, do tamanho de cabeças de alfinete, sobre uma base eritematosa, ocorrendo predominantemente no tronco, áreas intertriginosas e flexurais, com raro envolvimento da mucosa, principalmente oral. Febre, leucocitose no sangue periférico e eosinofilia leve são características associadas. Ocasionalmente ocorre envolvimento sistémico, com disfunção hepática, renal ou pulmonar. O início geralmente ocorre 1-12 dias após a administração do medicamento causal e é mais frequentemente associado a antibióticos beta-lactâmicos, macrolidos (incluindo pristinamicina e clindamicina), diltiazem, terbinafina, hidroxicloroquina, mas outros medicamentos têm sido implicados. A histologia revela pústulas espongiformes, subcórneas e/ou intraepidérmicas, mas esse padrão não é específico (o mesmo na psoríase pustulosa)."},"pl":{"name":"Ostra uogólniona osutka krostkowa","synonyms":["AGEP"],"definition":"Rzadka reakcja nadwrażliwości charakteryzująca się szybkim rozwojem licznych, niepęcherzykowych, sterylnych krost wielkości główki od szpilki na podłożu rumieniowym, występujących głównie na tułowiu, w zgięciach i okolicach podatnych na powstawanie zmian wyprzeniowych, z rzadkim zajęciem błony śluzowej, głównie jamy ustnej. Towarzyszy jej gorączka, leukocytoza i łagodna eozynofilia. Czasami rozwija się choroba ogólnoustrojowa z dysfunkcją wątroby, nerek lub płuc. Objawy pojawiają się zwykle 1-12 dni po podaniu leku sprawczego i są najczęściej związane z zastosowaniem antybiotyków beta-laktamowych, makrolidów (w tym pristynamycyny i klindamycyny), diltiazemu, terbinafiny, (hydroksy)chlorochiny, ale także wielu innych leków. W obrazie histologicznym widoczne są gąbczaste krosty umiejscowione pod warstwą rogową i/lub śródnaskórkowo, ale ten wzorzec zmian nie jest specyficzny (tak samo jak w łuszczycy krostkowej)."},"cs":{"name":"Akutní generalizovaná exantematózní pustulóza","synonyms":["AGEP","Pustulózní poléková erupce","Toxická pustuloderma"]},"uk":{"name":"Гострий генералізований екзантематозний пустульоз","synonyms":["AGEP","Гнійничкові медикаментозні висипання","Токсична пустульодермія"],"definition":"A rare hypersensitivity reaction characterized by the rapid development of numerous, nonfollicular, sterile, pinhead-sized pustules on an erythematous base, predominantly occurring on the trunk, intertriginous and flexural areas, with rare, mostly oral, mucosal involvement. Fever, peripheral blood leukocytosis, and mild eosinophilia are accompanying features. Systemic involvement, with hepatic, renal or pulmonary dysfunction, occasionally occurs. Onset usually occurs 1-12 days after administration of the causal medication and is most frequently associated with beta&#8208;lactam antibiotics, macrolides (including pristinamycin and clindamycin), diltiazem, terbinafine, (hydroxy&#8208;)chloroquine but many other medications have also been implicated. Histology reveals spongiform, subcorneal and/or intraepidermal, pustules but this pattern is not specific (same in pustular psoriasis)."},"zh":{"name":"急性泛发性发疹性脓疱病","synonyms":["脓疱性药疹"],"definition":"一种罕见的中毒性皮肤病，其特征是在水肿和红斑基础上迅速形成大量非滤泡状针尖样无菌脓疱，主要发生在躯干、肌腱间和弯曲部位，口腔粘膜少有受累。伴急性发热（ inf 38 sup U+2591 inf C）、外周血白细胞升高和轻度嗜酸性粒细胞升高。偶发全身性病变，伴有肝、肾或肺功能不全。组织学表现为典型的海绵状、角膜下和/或表皮内脓疱，真皮乳头状水肿明显，血管周明显的中性粒细胞及肺泡内或皮内嗜酸性粒细胞炎性浸润。"},"tr":{"name":"Akut jeneralize ekzantematöz püstüloz","synonyms":["AGEP","Toksik püstüloderma","Püstüler ilaç erupsiyonu"]}},"xrefs":[{"source":"ICD-10","code":"L27.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EH67.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"254592445"},{"source":"MONDO","code":"0017384","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D056150","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0877055","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10048799","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":["Multigenic/multifactorial","Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.3,"area":"Worldwide","status":"Not yet validated","source":"11168761[PMID]_ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.035,"area":"Israel","status":"Validated","source":"18669133[PMID]"},{"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":"2023-04-13","source":"23855377[PMID]_28084022[PMID]_34409363[PMID]_32926975[PMID]_35187690[PMID]_35054090[PMID]","terms":[{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032022","label":"Eosinophilic dermal infiltration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001880","label":"Increased total eosinophil count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011897","label":"Neutrophilia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040189","label":"Scaling skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100792","label":"Acantholysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100825","label":"Cheilitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001396","label":"Cholestasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000282","label":"Facial edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031236","label":"Predominantly dermal neutrophilic infiltrate","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200037","label":"Skin vesicle","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000509","label":"Conjunctivitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000953","label":"Hyperpigmentation of the skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Very rare (<4-1%)","criteria":null}]}}