{"orpha":79481,"name":"Pemphigus foliaceus","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare superficial pemphigus disease characterized by multiple, pruritic, scaly, crusted cutaneous erosions, with flaky circumscribed patches, localized mostly on the face, scalp, trunk and extremities, often presenting an erythematous base. Mucosal involvement is rarely observed.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-79481/","orphanet_url":"https://www.orpha.net/en/disease/detail/79481","names":{"fr":{"name":"Pemphigus foliacé","definition":"Pemphigus superficiel rare caractérisé par de multiples érosions cutanées prurigineuses, squameuses et croûteuses, ainsi que par des plaques circonscrites d'aspect floconneux, localisées principalement sur le visage, le cuir chevelu, le tronc et les extrémités, souvent sur fond érythémateux. L'atteinte des muqueuses est rare."},"de":{"name":"Pemphigus foliaceus","definition":"Eine seltene superfizielle Pemphiguserkrankung, die durch multiple, juckende, schuppende, verkrustete Hauterosionen mit schuppigen, umschriebenen Flecken gekennzeichnet ist, die meist im Gesicht, auf der Kopfhaut, am Rumpf und an den Extremitäten lokalisiert sind und oft einen erythematösen Untergrund aufweisen. Eine Beteiligung der Schleimhäute wird nur selten beobachtet."},"es":{"name":"Pénfigo foliáceo","definition":"Es un tipo de pénfigo superficial poco frecuente caracterizado por múltiples erosiones cutáneas descamativas, costrosas y pruriginosas, y placas descamativas circunscritas, localizadas principalmente en la cara, cuero cabelludo, tronco y extremidades, que con frecuencia presentan una base eritematosa. Excepcionalmente se observa afectación de las mucosas."},"it":{"name":"Pemfigo foliaceo","definition":"Il pemfigo foliaceo è un pemfigo superficiale raro, caratterizzato da erosioni cutanee multiple pruriginose, desquamative e crostose, associate a macchie desquamative circoscritte, che interessano per lo più il viso, il cuoio capelluto, il tronco e gli arti, spesso con base eritematosa. Le mucose sono raramente colpite."},"nl":{"name":"Pemphigus foliaceus","definition":"Een zeldzame, oppervlakkige pemfigus, gekarakteriseerd door multipele, pruritische, schilferende, crusteuze huiderosies met vlokkige, begrensde plekken, meestal gesitueerd op aangezicht, scalp, romp en ledematen, en vaak op een erythemateuze basis. Betrokkenheid van slijmvlies wordt zelden waargenomen."},"pt":{"name":"Pênfigo foliáceo"},"pl":{"name":"Pęcherzyca liściasta","definition":"*Pęcherzyca liściasta jest rzadką, powierzchowną pęcherzycą, która charakteryzuje się licznymi, swędzącymi, łuszczącymi się nadżerkami pokrytymi strupami, z łuskowatymi, i dobrze odgraniczonymi zmianami skórnymi, zlokalizowanymi głównie na twarzy, skórze głowy, tułowiu i kończynach, często na rumieniowatym podłożu. Rzadko obserwuje się zajęcie błony śluzowej."},"cs":{"name":"Pemphigus foliaceus"},"uk":{"name":"Пемфігоїд рубцевий","definition":"A rare superficial pemphigus disease characterized by multiple, pruritic, scaly, crusted cutaneous erosions, with flaky circumscribed patches, localized mostly on the face, scalp, trunk and extremities, often presenting an erythematous base. Mucosal involvement is rarely observed."},"zh":{"name":"落叶型天疱疮"},"tr":{"name":"Pemfigus foliaceus"}},"xrefs":[{"source":"ICD-10","code":"L10.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB40.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"24246260"},{"source":"MONDO","code":"0019324","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0263313","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7354","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10057069","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":46485,"name":"Superficial pemphigus","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.001,"area":"Germany","status":"Validated","source":"27456755[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-19","source":"31378971[PMID]_29479654[PMID]_30762246[PMID]_29267466[PMID]","terms":[{"hpo":"HP:0100792","label":"Acantholysis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001965","label":"Abnormality of the scalp","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007473","label":"Crusting erythematous dermatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025474","label":"Erythematous plaque","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:0200041","label":"Skin erosion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011830","label":"Abnormal oral mucosa morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001019","label":"Erythroderma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200037","label":"Skin vesicle","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025528","label":"Annular cutaneous lesion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0004377","label":"Hematological neoplasm","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008069","label":"Neoplasm of the skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003765","label":"Psoriasiform dermatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025527","label":"Serpiginous cutaneous lesion","frequency":"Very rare (<4-1%)","criteria":null}]}}