{"orpha":538866,"name":"Pustular pyoderma gangrenosum","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare subtype of pyoderma gangrenosum characterized by multiple painful, sterile pustules with a surrounding erythematous halo, predominantly occurring on the trunk and extensor surfaces of the limbs, and potentially persisting for months. Histopathology shows a dermal neutrophilic infiltrate and subcorneal neutrophilic micropustules. The condition is commonly associated with inflammatory bowel disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-538866/","orphanet_url":"https://www.orpha.net/en/disease/detail/538866","names":{"fr":{"name":"Pyoderma gangrenosum, type pustuleux","definition":"Sous-type rare de pyoderma gangrenosum caractérisée par de multiples pustules douloureuses et stériles entourées d'un halo érythémateux, apparaissant principalement sur le tronc et les surfaces d'extension des membres, et pouvant persister pendant des mois. L'histopathologie révèle un infiltrat neutrophilique dermique et des micropustules neutrophiliques sous-cornées. La maladie est généralement associée à une maladie inflammatoire de l'intestin."},"de":{"name":"Pyoderma gangraenosum, pustulöses","definition":"Ein seltener Subtyp der Pyoderma gangrenosum, der durch multiple schmerzhafte, sterile Pusteln mit einem umgebenden erythematösen Halo gekennzeichnet ist, vorwiegend am Rumpf und an den Streckseiten der Gliedmaßen auftritt und möglicherweise monatelang bestehen bleibt. Die Histopathologie zeigt ein dermales neutrophiles Infiltrat und subkorneale neutrophile Mikropusteln. Die Erkrankung wird häufig mit entzündlichen Darmerkrankungen in Verbindung gebracht."},"es":{"name":"Pioderma gangrenoso pustuloso","definition":"Es un subtipo poco frecuente de pioderma gangrenoso caracterizado por múltiples pústulas estériles dolorosas con un halo eritematoso periférico, que ocurre predominantemente en el tronco y las superficies extensoras de las extremidades, pudiendo persistir durante meses. La histopatología muestra un infiltrado neutrofílico dérmico y micropústulas neutrofílicas subcorneales. El trastorno está asociado con frecuencia a una enfermedad inflamatoria intestinal."},"it":{"name":"Pioderma gangrenoso pustoloso","definition":"Si tratta di un sottotipo raro di pioderma gangrenoso, caratterizzato da pustole multiple sterili e dolorose, circondate da un alone eritematoso, localizzate prevalentemente sul tronco e sulle superfici estensorie degli arti, che possono persistere per mesi. L'esame istopatologico mostra un infiltrato neutrofilo nel derma e micropustole neutrofile subcorneali. In genere si associa alla malattia infiammatoria intestinale"},"nl":{"name":"Pustuleuze pyoderma gangrenosum","definition":"Een zeldzaam subtype van pyoderma gangrenosum, gekarakteriseerd door multipele pijnlijke, steriele pustels die omgeven zijn door een erythemateuze ring, hoofdzakelijk voorkomen op romp en strekzijde van ledematen, en mogelijk maandenlang aanhouden. Histopathologie toont een dermaal neutrofiel infiltraat en subcorneale neutrofiele micropustels. De aandoening is doorgaans geassocieerd met inflammatoire darmziekte."},"pt":{"name":"Pioderma gangrenoso pustular"},"pl":{"name":"Pioderma krostkowa zgorzelinowa"},"cs":{"name":"Pustulární pyoderma gangrenosum"},"uk":{"name":"Гнійничкова гангренозна піодермія","definition":"A rare subtype of pyoderma gangrenosum characterized by multiple painful, sterile pustules with a surrounding erythematous halo, predominantly occurring on the trunk and extensor surfaces of the limbs, and potentially persisting for months. Histopathology shows a dermal neutrophilic infiltrate and subcorneal neutrophilic micropustules. The condition is commonly associated with inflammatory bowel disease."},"zh":{"name":"脓疱性坏疽性脓皮病"},"tr":{"name":"Püstüler piyoderma gangrenozum"}},"xrefs":[{"source":"ICD-10","code":"L88","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB21","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1812847260"},{"source":"MONDO","code":"0035236","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681317","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":48104,"name":"Pyoderma gangrenosum","classifications":[156,187,196]}],"children":[],"genes":[{"symbol":"PTPN6","name":"protein tyrosine phosphatase non-receptor type 6","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"21406173[PMID]","hgnc":"9658","ensembl":"ENSG00000111679","omim":"176883","loci":["12p13.31"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25213386[PMID]"},{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Italy","status":"Validated","source":"ORPHANET_29877043[PMID]"}],"phenotypes":null}