{"orpha":538863,"name":"Classic pyoderma gangrenosum","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Ulcerative pyoderma gangrenosum"],"definition":"A rare subtype of pyoderma gangrenosum disease characterized by rapidly progressive, single or multiple, painful, aseptic ulcers which present overhanging, violaceous and undermined borders, surrounding induration and erythema, and granulation tissue (occasionally necrotic tissue and/or a purulent exudate) at the base, mainly affecting the legs (but other body surfaces may also be involved), leading to chronic ulcerations and often regressing with cribriform mutilating scars. The disease presents a chronic relapsing course and systemic features (e.g. fever, malaise, arthralgia, myalgia) may be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-538863/","orphanet_url":"https://www.orpha.net/en/disease/detail/538863","names":{"fr":{"name":"Pyoderma gangrenosum, type classique","synonyms":["Pyoderma gangrenosum, type ulcéreux"],"definition":"Sous-type rare de pyoderma gangrenosum caractérisé par des ulcères aseptiques douloureux, isolés ou multiples, rapidement progressifs, dont les bords sous-minés de couleur violacée forment une saillie autour de la lésion d'aspect érythémateux et induré, avec apparition d'un tissu de granulation (occasionnellement un tissu nécrotique et/ou un exsudat purulent) à la base de l'ulcère. Principalement localisés sur les jambes (bien que d'autres zones corporelles puissent être atteintes), ces ulcères deviennent chroniques et régressent souvent en laissant d'importantes cicatrices cribriformes. Potentiellement associée à des signes systémiques (fièvre, malaise, arthralgie, myalgie), la maladie évolue de manière chronique et récurrente."},"de":{"name":"Pyoderma gangraenosum, klassisches","synonyms":["Pyoderma gangraenosum, ulzeratives"],"definition":"Eine seltener Subtyp des Pyoderma gangrenosum, der durch schnell fortschreitende, einzelne oder multiple, schmerzhafte, aseptische Ulzerationen gekennzeichnet ist. Das betroffene Gewebe präsentiert sich mit überhängender, violetter und unscharfer Abgrenzung, einer umgebenden Verhärtung und Rötung sowie Granulationsgewebe (gelegentlich nekrotisches Gewebe und/oder ein eitriges Exsudat) an der Basis. Es betrifft häufig die Beine (aber es können auch andere Körperoberflächen betroffen sein) und kann zu chronischen Ulzerationen führen, die sich oft unter Ausprägung von spindelförmigen, mutilierenden Narben zurückbilden. Die Erkrankung zeigt einen chronisch-rezidivierenden Verlauf und kann mit systemischen Merkmalen (z. B. Fieber, Unwohlsein, Arthralgie, Myalgie) einhergehen."},"es":{"name":"Pioderma gangrenoso clásico","synonyms":["Pioderma gangrenoso ulcerativo"],"definition":"Es un subtipo de pioderma gangrenoso poco frecuente, caracterizado por úlceras asépticas, dolorosas, aisladas o múltiples, rápidamente progresivas, que presentan bordes sobreelevados, violáceos y dentados, induración y eritema periféricos, con tejido de granulación (ocasionalmente tejido necrótico y/o exudado purulento) en la base, que afecta principalmente a las piernas (aunque también puede afectar a otras partes del cuerpo), que conducen a úlceras crónicas que con frecuencia dan lugar a cicatrices cribiformes mutilantes. La enfermedad presenta un curso crónico recurrente y puede asociar hallazgos sistémicos (p. ej. fiebre, malestar general, artralgia, mialgia)."},"it":{"name":"Pioderma gangrenoso classico","definition":"Si tratta di un sottotipo raro di pioderma gangrenoso, caratterizzato da ulcere asettiche dolorose, singole o multiple, a progressione rapida, che presentano bordi sporgenti violacei e male delineati, circostante indurimento ed eritema, e tessuto di granulazione (occasionalmente tessuto necrotico e/o essudato purulento) alla base. Le lesioni si localizzano soprattutto sulle gambe (ma possono interessare anche altre aree del corpo) ed esitano in ulcere croniche, che spesso regrediscono lasciando cicatrici cribriformi mutilanti. La malattia, il cui decorso è cronico e recidivante, può associarsi a sintomi sistemici (ad es. febbre, malessere, artralgia, mialgia)."},"nl":{"name":"Klassieke pyoderma gangrenosum","synonyms":["Ulceratieve pyoderma gangrenosum"],"definition":"Een zeldzaam subtype van pyoderma gangrenosum gekarakteriseerd door snel progressieve, solitaire of multipele, pijnlijke, aseptische zweren met overhangende, violette en ondermijnde randen rond induratie en erytheem, en granulatieweefsel (occasioneel necrotisch weefsel en/of een purulent exsudaat) aan de basis, die voornamelijk de benen treffen (maar andere oppervlakken van het lichaam kunnen ook betrokken zijn), en die leiden tot chronische ulceraties en vaak regressie tot cribriforme mutilerende littekens vertonen. De ziekte vertoont een chronisch recurrent verloop en systemische kenmerken (e.g. koorts, malaise, artralgie, myalgie) kunnen geassocieerd zijn."},"pt":{"name":"Pioderma gangrenoso clássico"},"pl":{"name":"Klasyczna piodermia zgorzelinowa","synonyms":["Ulcerative pyoderma gangrenosum"]},"cs":{"name":"Klasická pyoderma gangrenosum","synonyms":["Ulcerózní pyoderma gangrenosum"]},"uk":{"name":"Класична гангренозна піодермія","synonyms":["Виразкова гангренозна піодермія"],"definition":"A rare subtype of pyoderma gangrenosum disease characterized by rapidly progressive, single or multiple, painful, aseptic ulcers which present overhanging, violaceous and undermined borders, surrounding induration and erythema, and granulation tissue (occasionally necrotic tissue and/or a purulent exudate) at the base, mainly affecting the legs (but other body surfaces may also be involved), leading to chronic ulcerations and often regressing with cribriform mutilating scars. The disease presents a chronic relapsing course and systemic features (e.g. fever, malaise, arthralgia, myalgia) may be associated."},"zh":{"name":"经典坏疽性脓皮病","synonyms":["溃疡性坏疽性脓皮病"],"definition":"一种罕见的坏疽性脓皮病亚型，其特征是快速进展的单发或多发的疼痛性无菌性溃疡，呈现悬垂的、紫罗兰色和破坏性边界，周围有硬结和红斑，基底有肉芽组织（偶有坏死组织和（或）脓性渗出物），主要累及腿部（但其他体表也可受累），导致慢性溃疡，常退行性地出现筛状残缺性疤痕。本病呈慢性复发病程，可能伴有全身性特征（如发热、乏力、关节痛、肌痛）。"},"tr":{"name":"Klasik piyoderma gangrenozum","synonyms":["Ülseratif 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":"1951293028"},{"source":"MONDO","code":"0035235","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680157","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":"1-9 / 1 000 000","value":0.0,"area":"Italy","status":"Validated","source":"ORPHANET_29877043[PMID]"}],"phenotypes":null}