{"orpha":63275,"name":"Pemphigoid gestationis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Gestational pemphigoid"],"definition":"A rare autoimmune bullous skin disease characterized by pruritus with or without polymorphic skin eruption, affecting pregnant women typically during the second and third trimester.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-63275/","orphanet_url":"https://www.orpha.net/en/disease/detail/63275","names":{"fr":{"name":"Pemphigoïde gravidique","synonyms":["Pemphigoïde de la grossesse","Pemphigoïde gestationnelle"],"definition":"Dermatite bulleuse auto-immune rare caractérisée par un prurit avec ou sans éruption cutanée polymorphe, affectant les femmes enceintes généralement au cours du deuxième et troisième trimestre de grossesse."},"de":{"name":"Pemphigoid gestationis","synonyms":["Schwangerschafts-Pemphigoid"],"definition":"Eine seltene bullöse Autoimmunerkrankung der Haut, die durch Juckreiz mit oder ohne polymorphe Hauteruptionen gekennzeichnet ist und schwangere Frauen typischerweise im zweiten und dritten Trimester betrifft."},"es":{"name":"Penfigoide gestationis","synonyms":["Herpes gestationis","Penfigoide gestacional"],"definition":"Es una enfermedad cutánea ampollosa autoinmune poco frecuente caracterizada por prurito, con o sin erupciones cutáneas polimórficas, que afecta a mujeres embarazadas típicamente durante el segundo y tercer trimestre de gestación. Las erupciones cutáneas pueden incluir pápulas y placas eritematosas, lesiones de tipo eritema multiforme o eccematosas, papulovesículas y ampollas, y afectan típicamente al ombligo, el abdomen y las extremidades."},"it":{"name":"Pemfigoide gestazionale","synonyms":["Pemfigoide gravidico"],"definition":"Il pemfigoide gestazionale (PG) è una malattia cutanea bollosa autoimmune rara, caratterizzata da prurito con o senza eruzioni cutanee polimorfe, che colpisce le donne gravide di solito nel secondo e terzo trimestre. Le eruzioni cutanee comprendono le papule e le placche eritematose, le lesioni eczematose o simil-eritema multiforme, le vescicole papulose e le bolle, che in genere si localizzano sull'ombelico, sull'addome e sugli arti."},"nl":{"name":"Pemphigoid gestationis","synonyms":["Gestationeel pemfigoïd"],"definition":"Een zeldzame, auto-immune bulleuze huidziekte, gekarakteriseerd door pruritus met of zonder polymorfe huiduitslag die zwangere vrouwen treft, doorgaans in het tweede of derde trimester."},"pt":{"name":"Penfigoide gestacional"},"pl":{"name":"Pemfigoid ciężarnych","synonyms":["Gestational pemphigoid"]},"cs":{"name":"Gestační pemphigoid","synonyms":["Gestační pemfigoid"]},"uk":{"name":"Гестаційний пемфігоїд","synonyms":["Гестаційний пемфігоїд"],"definition":"A rare autoimmune bullous skin disease characterized by pruritus with or without polymorphic skin eruption, affecting pregnant women typically during the second and third trimester."},"zh":{"name":"妊娠性类天疱疮","synonyms":["妊娠性类天疱疮"]},"tr":{"name":"Pemfigoid gestationis","synonyms":["Pemfigoid gestasyones"]}},"xrefs":[{"source":"ICD-10","code":"O26.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"JA65.10","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1405755890"},{"source":"MONDO","code":"0006558","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D006559","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0019343","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6497","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10019939","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":["Adolescent","Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.2,"area":"Germany","status":"Validated","source":"19170813[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.05,"area":"France","status":"Validated","source":"7826096[PMID]_23237497[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.18,"area":"Kuwait","status":"Validated","source":"15569006[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.693,"area":"Germany","status":"Validated","source":"27456755[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":"25178359[PMID]","terms":[{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001622","label":"Premature birth","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200037","label":"Skin vesicle","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001508","label":"Failure to thrive","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001511","label":"Intrauterine growth retardation","frequency":"Frequent (79-30%)","criteria":null}]}}