{"orpha":64745,"name":"Pruritic urticarial papules and plaques of pregnancy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PUPPP","Polymorphic eruption of pregnancy"],"definition":"A rare skin disease characterized by urticarial papules and plaques with severe pruritus mainly on the abdomen, buttocks, and proximal thighs. The condition usually develops during the third trimester of the first pregnancy, although presentation in the postpartum period, which may also feature other types of skin lesions, has been described in some cases. The symptoms generally resolve within few weeks.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-64745/","orphanet_url":"https://www.orpha.net/en/disease/detail/64745","names":{"fr":{"name":"Plaques et papules prurigineuses urticariennes de la grossesse","synonyms":["Eruption polymorphe de la grossesse","PUPPP"],"definition":"Maladie de peau rare caractérisée par des papules et des plaques urticariennes avec un prurit sévère principalement sur l'abdomen, les fesses et les cuisses. La maladie apparaît généralement au cours du troisième trimestre de la première grossesse, bien que, dans certains cas décrits, elle puisse se manifester au cours de la période post-partum, par d'autres types de lésions cutanées. Les signes cliniques disparaissent généralement en quelques semaines."},"de":{"name":"Pruritische urtikarielle Papeln und Plaques in der Schwangerschaft","synonyms":["PUPPP","Schwangerschaftsdermatose, polymorphe"],"definition":"Eine seltene Hauterkrankung, die durch urtikarielle Papeln und Plaques mit starkem Juckreiz hauptsächlich am Bauch, Gesäß und den proximalen Oberschenkeln gekennzeichnet ist. Die Erkrankung entwickelt sich in der Regel im dritten Trimester der ersten Schwangerschaft, obwohl in einigen Fällen auch ein Auftreten im Wochenbett beschrieben wurde, das auch andere Arten von Hautläsionen aufweisen kann. Die Symptome klingen im Allgemeinen innerhalb weniger Wochen ab."},"es":{"name":"Pápulas urticariales pruriginosas y placas de embarazo","synonyms":["Erupción polimórfica del embarazo"],"definition":"Es una enfermedad cutánea poco frecuente caracterizada por la presencia de pápulas y placas urticariales acompañadas de prurito intenso, principalmente en el abdomen, las nalgas y la parte proximal de los muslos. El trastorno se desarrolla típicamente durante el tercer trimestre del primer embarazo, aunque en algunos casos se ha descrito la presentación durante el puerperio, que también puede asociar otros tipos de lesiones cutáneas. Por lo general, los síntomas se resuelven en pocas semanas."},"it":{"name":"Papule e placche orticarioidi pruriginose della gravidanza","synonyms":["Eruzione polimorfa della gravidanza"],"definition":"Si tratta di una malattia rara della cute, caratterizzata da papule e placche orticarioidi associate a prurito intenso, localizzate per lo più sull'addome, sulle natiche e sulla porzione prossimale delle cosce. Di solito la malattia esordisce nel terzo trimestre della prima gravidanza, ma sono stati documentati casi di esordio nel post partum, in cui sono stati osservati anche altri tipi di lesioni cutanee. In genere i sintomi scompaiono nell'arco di qualche settimana."},"nl":{"name":"Pruritische urticariële papels en plaques bij zwangerschap","synonyms":["PUPPP","Polymorfe eruptie bij zwangerschap"],"definition":"Een zeldzame huidziekte, gekarakteriseerd door urticariële papels en plaques met ernstige pruritus, vooral op abdomen, billen, en proximale dijen. De aandoening ontwikkelt meestal tijdens het derde trimester van de eerste zwangerschap, hoewel in enkele gevallen presentatie in de postpartum periode, wanneer ook andere soorten huidlaesies kunnen voorkomen, werd beschreven. De symptomen verdwijnen doorgaans binnen enkele weken."},"pt":{"name":"Pápulas e placas pruriginosa urticárias da gravidez","synonyms":["Erupção polimórfica da gravidez"]},"pl":{"name":"Swędzące grudki i ogniska pokrzywkowe w przebiegu ciąży","synonyms":["Wielopostaciowe osutki ciążowe"]},"cs":{"name":"Pruritické urtikariální papuly a plaky v graviditě","synonyms":["Polymorfní erupce v těhotenství"]},"uk":{"name":"Сверблячі кропивяні папули та бляшки при вагітності","synonyms":["Поліморфні висипання під час вагітності","PUPPP"],"definition":"A rare skin disease characterized by urticarial papules and plaques with severe pruritus mainly on the abdomen, buttocks, and proximal thighs. The condition usually develops during the third trimester of the first pregnancy, although presentation in the postpartum period, which may also feature other types of skin lesions, has been described in some cases. The symptoms generally resolve within few weeks."},"zh":{"name":"妊娠瘙痒性荨麻疹性丘疹及斑块","synonyms":["妊娠性多形疹"]},"tr":{"name":"Gebeliğin döküntülü kaşıntısı","synonyms":["PUPPP","Gebelikte polimorfik patlama"]}},"xrefs":[{"source":"OMIM","code":"178995","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"O26.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"JA65.12","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"968694549"},{"source":"MONDO","code":"0008353","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C535817","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0269680","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9635","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10066100","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":163637,"name":"Rare disorder related with pregnancy, childbirth and puerperium","classifications":[205]},{"orpha":79384,"name":"Rare urticaria","classifications":[187,212]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_27960565[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-07-09","source":"9843004[PMID]_30969522[PMID]_22909357[PMID]_31903969[PMID]","terms":[{"hpo":"HP:0030350","label":"Erythematous papule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001065","label":"Striae distensae","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030351","label":"Urticarial plaque","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025474","label":"Erythematous plaque","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004324","label":"Increased body weight","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001036","label":"Parakeratosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030898","label":"Pruritis on abdomen","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200037","label":"Skin vesicle","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031538","label":"Abnormal dermoepidermal junction morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100872","label":"Abnormality of the plantar skin of foot","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001041","label":"Facial erythema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031248","label":"Palmar pruritus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030899","label":"Pruritis on hand","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030900","label":"Pruritus on foot","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000964","label":"Eczematoid dermatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025493","label":"Palmoplantar erythema","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030901","label":"Pruritis on breast","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031541","label":"Linear C3 deposits along the epidermal basement membrane zone","frequency":"Excluded (0%)","criteria":null},{"hpo":"HP:0031540","label":"Linear IgG deposits along the epidermal basement membrane zone","frequency":"Excluded (0%)","criteria":null}]}}