{"orpha":79479,"name":"Pemphigus vegetans","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-79479/","orphanet_url":"https://www.orpha.net/en/disease/detail/79479","names":{"fr":{"name":"Pemphigus végétant","definition":"Dermatite bulleuse auto-immune rare, caractérisée par des bulles cutanéo-muqueuses avec érosion ultérieure et formation de plaques végétatives, affectant principalement les zones intertrigineuses et la muqueuse buccale. Peut se présenter sous deux formes cliniques : le type Hallopeau, qui présente une évolution indolente et végétante des pustules et une absence fréquente d'atteinte de la muqueuse buccale, et le type Neumann, qui prend une forme plus sévère et réfractaire avec une évolution végétante au cours d'une éruption de lésions vésiculobulleuses et une atteinte de la muqueuse buccale. L'analyse sérique révèle des anticorps dirigés contre la desmogléine 1 et 3."},"de":{"name":"Pemphigus vegetans","definition":"Eine bullöse Autoimmunerkrankung der Haut, die durch mukokutane Blasenbildung mit anschließender Erosion und Bildung vegetativer Plaques gekennzeichnet ist und vorwiegend intertriginöse Bereiche und die Mundschleimhaut betrifft. Es werden zwei klinische Formen der Krankheit unterschieden: der Hallopeau-Typ mit indolentem Verlauf, bei dem die Blasen als vegetative Plaques abheilen und die Mundschleimhaut häufig nicht betroffen ist, und der Neumann-Typ mit schwerem, refraktärem Verlauf, bei dem sich während des Ausbruchs vesikulobullöser Läsionen Vegetationen entwickeln und die Mundschleimhaut betroffen ist. Die Serumanalyse zeigt Antikörper gegen Desmoglein 1 und 3."},"es":{"name":"Pénfigo vegetativo","definition":"Es una enfermedad cutánea ampollosa autoinmune poco frecuente caracterizada por unas ampollas mucocutáneas con posterior erosión y formación de unas placas excrecentes, vegetantes, que afectan predominantemente a las áreas intertriginosas y a la mucosa oral. Se reconocen dos formas clínicas de la enfermedad: el tipo Hallopeau, que presenta un curso indolente con una cicatrización de las pústulas como placas vegetativas y frecuente preservación de la mucosa oral, y el tipo Neumann, que sigue un curso más grave y refractario con el desarrollo de vegetaciones durante una erupción de lesiones vesículo-ampollosas y una afectación de la mucosa oral. El análisis de suero revela anticuerpos contra la desmogleína 1 y 3."},"it":{"name":"Pemfigo vegetante","definition":"Si tratta di una malattia cutanea bollosa autoimmune rara, caratterizzata da bolle muco-cutanee associate all'erosione e alla formazione di placche vegetative, che interessano per lo più le zone intertriginose e la mucosa orale. Si conoscono due forme cliniche della malattia: il tipo Hallopeau, caratterizzato da un decorso silente con pustole che guariscono così come le placche vegetative, spesso senza interessamento della mucosa orale, e il tipo Neumann, caratterizzato da un decorso più grave e refrattario, e dall'insorgenza di lesioni vegetative nel quadro di un'eruzione di lesioni vescicolo-bollose, con interessamento della mucosa orale. Gli esami di laboratorio sul siero mostrano anticorpi diretti contro la desmogleina 1 e 3."},"nl":{"name":"Pemphigus vegetans","definition":"Een zeldzame auto-immune bulleuze aandoening van huid, gekarakteriseerd door mucocutane bullae gevolgd door erosie en vorming van vegetatieve plaques, voornamelijk op intertrigineuze gebieden en mondslijmvlies. Er worden twee klinische vormen van de ziekte onderscheiden: Hallopeau-type, dat een indolent verloop vertoont met pustels die helen als vegetatieve plaques en vaak zonder betrokkenheid van mondslijmvlies, en Neumann-type, dat een ernstiger, refractair verloop vertoont met vegetaties die ontwikkelen tijdens een eruptie van vesiculobulleuze laesies en betrokkenheid van mondslijmvlies. Analyse van serum toont antilichamen tegen desmogleïne 1 en 3."},"pt":{"name":"Pênfigo vegetante"},"pl":{"name":"Pęcherzyca bujająca"},"cs":{"name":"Pemphigus vegetans"},"uk":{"name":"Пемфігус вегетуючий","definition":"A rare autoimmune bullous skin disease characterized by mucocutaneous bullae with subsequent erosion and formation of vegetative plaques, predominantly affecting intertriginous areas and the oral mucosa. Two clinical forms of the disease are recognized: the Hallopeau type, which presents an indolent course with pustules healing as vegetative plaques and frequent lack of involvement of the oral mucosa, and the Neumann type, which takes a more severe, refractory course with vegetations developing during an eruption of vesiculobullous lesions and involvement of the oral mucosa. Serum analysis reveals antibodies against desmoglein 1 and 3."},"zh":{"name":"增殖型天疱疮"},"tr":{"name":"Pemfigus vejetans"}},"xrefs":[{"source":"ICD-10","code":"L10.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EB40.0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1977394957"},{"source":"MONDO","code":"0019322","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0263316","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10057053","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79669,"name":"Autoimmune bullous skin disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.391,"area":"Germany","status":"Validated","source":"27456755[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2026-05-19","source":"31365654[PMID]_31424813[PMID]_260510641[PMID]","terms":[{"hpo":"HP:4000013","label":"Anti-desmoglein-1 antibody positivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:4000014","label":"Anti-desmoglein-3 antibody positivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000159","label":"Abnormal lip morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030809","label":"Abnormal tongue morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:6000448","label":"Anti-desmocollin antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:6000956","label":"Exuberant granulation tissue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000962","label":"Hyperkeratosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033802","label":"Intra-epidermal blistering","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032007","label":"Maceration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200097","label":"Oral mucosal blisters","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033700","label":"Papulovesicular eruption","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200035","label":"Skin plaque","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010280","label":"Stomatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012500","label":"Verrucous papule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033697","label":"Vesicular eruption","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001597","label":"Abnormal nail morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025757","label":"Abnormal nail surface","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001808","label":"Fragile nails","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004395","label":"Malnutrition","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025088","label":"Onychomadesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001818","label":"Paronychia","frequency":"Occasional (29-5%)","criteria":null}]}}