{"orpha":641390,"name":"PsAPASH syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Psoriatic arthritis-pyoderma gangrenosum-acne-hidradenitis suppurativa syndrome"],"definition":"A rare autoinflammatory syndrome characterized by the combination of psoriatic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis (which, in addition to axillae and inguinal folds, can be observed in other areas, such as the buttocks or labia majora).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-641390/","orphanet_url":"https://www.orpha.net/en/disease/detail/641390","names":{"fr":{"name":"Syndrome PsAPASH","synonyms":["Syndrome d'arthrite psoriasique-pyoderma gangrenosum-acné-hidradénite suppurée"],"definition":"Syndrome auto-inflammatoire rare caractérisé par l'association d'un rhumatisme psoriasique, d'un pyoderma gangrenosum, d'une acné et d'une hidradénite suppurée (qui, outre les aisselles et les plis inguinaux, peut être observée à d'autres endroits, tels que les fesses ou les grandes lèvres)."},"de":{"name":"PsAPASH-Syndrom","synonyms":["Psoriasis-Arthritis-Pyoderma gangrenosum-Akne-Hidradenitis suppurativa-Syndrom"],"definition":"Ein seltenes autoinflammatorisches Syndrom, das durch eine Kombination aus Psoriasis-Arthritis, Pyoderma gangraenosum, Akne und suppurativer Hidradenitis (die neben den Achselhöhlen und Leistenbeugen auch in anderen Bereichen wie dem Gesäß oder den großen Schamlippen auftreten kann) gekennzeichnet ist."},"es":{"name":"Síndrome PsAPASH","synonyms":["Síndrome de artritis psoriásica-pioderma gangrenoso-acné-hidradenitis supurativa"],"definition":"Es un síndrome autoinflamatorio poco frecuente caracterizado por la combinación de artritis psoriásica, pioderma gangrenoso, acné e hidradenitis (presente en los glúteos o labios mayores, además de axilas y pliegues inguinales, entre otros)."},"it":{"name":"Sindrome PsAPASH","synonyms":["Sindrome artrite psoriasica- pioderma gangrenoso-acne-idrosadenite suppurativa"]},"nl":{"name":"PsAPASH-syndroom","synonyms":["Artritis psoriatica - pyoderma gangrenosum - acne - hidradenitis suppurativa-syndroom","Psoriasisartritis - pyoderma gangrenosum - acne - hidradenitis suppurativa-syndroom"],"definition":"Een zeldzaam auto-inflammatoir syndroom, gekenmerkt door de combinatie van arthritis psoriatica, pyoderma gangrenosum, acne, en hidradenitis suppurativa (die bovenop oksels en liesstreek kunnen waargenomen worden in andere gebieden zoals billen en grote schaamlippen)."},"pt":{"name":"Síndrome PsAPASH","synonyms":["Síndrome de artrite psoriática-pioderma gangrenoso-acne-hidradenite supurativa"],"definition":"Síndrome autoinflamatória rara caracterizada pela combinação de artrite psoriática, pioderma gangrenoso, acne e hidradenite supurativa (que, além das axilas e pregas inguinais, pode ser observada em outras áreas, como nádegas ou grandes lábios)."},"pl":{"name":"Zespół PsAPASH","synonyms":["Psoriatic arthritis-pyoderma gangrenosum-acne-hidradenitis suppurativa syndrome"],"definition":"Rzadki zespół autozapalny charakteryzujący się połączeniem łuszczycowego zapalenia stawów, piodermii zgorzelinowej, trądziku i ropnego zapalenia gruczołów potowych (które oprócz pachwin i dołów pachowych dotyczy także innych okolic ciała, np. pośladków lub warg sromowych większych)."},"cs":{"name":"PsAPASH syndrom","synonyms":["Psoriatic arthritis-pyoderma gangrenosum-acne-hidradenitis suppurativa syndrome"]},"uk":{"name":"Синдром PsAPASH","synonyms":["Псоріатичний артрит - гангренозна піодермія - акне - гнійничковий синдром - гідраденіт"],"definition":"A rare autoinflammatory syndrome characterized by the combination of psoriatic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis (which, in addition to axillae and inguinal folds, can be observed in other areas, such as the buttocks or labia majora)."}},"xrefs":[{"source":"ICD-10","code":"L88","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB21","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0958257","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"958257","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5816786","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":653434,"name":"Autoinflammatory syndrome with acne and/or hidradenitis suppurativa","classifications":[187,196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":10.0,"area":"Worldwide","status":"Validated","source":"32942279[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"32942279[PMID]_ORPHANET"}],"phenotypes":null}