{"orpha":538872,"name":"Vegetative pyoderma gangrenosum","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Granulomatous pyoderma gangrenosum"],"definition":"A rare subtype of pyoderma gangrenosum disease characterized by a solitary, erythematous, ulcerated plaque, which lacks the violaceous border typically present in classic pyoderma gangrenosum, usually affecting individuals who are otherwise healthy. Histologically, the lesion presents a central layer containing neutrophilic inflamation, surrounded by a palisade of histiocytes, which are rimmed by a lymphocytic infiltrate. In comparison with the other variants of pyoderma gangrenosum, this subtype usually shows a good response to less aggressive treatments and underlying systemic disorders are less frequently associated. It is considered the most benign and uncommon clinical variant of pyoderma gangrenosum.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-538872/","orphanet_url":"https://www.orpha.net/en/disease/detail/538872","names":{"fr":{"name":"Pyoderma gangrenosum, type végétatif","synonyms":["Pyodermite granulomateuse superficielle"],"definition":"Sous-type rare de pyoderma gangrenosum caractérisé par une plaque unique, érythémateuse et ulcérée, dépourvue des bords violacés caractéristiques du type classique de pyoderma gangrenosum, et qui touche généralement des personnes en bonne santé. L'examen histologique met en évidence une lésion dont la couche centrale renferme une inflammation neutrophilique, entourée d'une palissade d'histiocytes, eux-mêmes bordés par un infiltrat lymphocytaire. Par rapport aux autres variantes du pyoderma gangrenosum, ce sous-type répond généralement bien à des traitements moins agressifs et est moins fréquemment associé à des maladies systémiques sous-jacentes. Il s'agit de la variante clinique de pyoderma gangrenosum la moins maligne et la moins fréquente."},"de":{"name":"Pyoderma gangraenosum, vegetatives","synonyms":["Pyoderma gangraenosum, granulomatöses"],"definition":"Eine seltener Subtyp des Pyoderma gangrenosum, der durch eine solitäre, erythematöse, ulzerierte Plaque gekennzeichnet ist und der gewöhnlich Personen betrifft, die ansonsten gesund sind. Im Gegensatz zur klassischen Form des Pyoderma gangrenosum fehlt der typische violette Rand. Histologisch weist die Läsion eine zentrale Schicht mit neutrophiler Entzündung auf, umgeben von einer Palisade aus Histiozyten, die von einem lymphozytären Infiltrat umrandet sind. Im Vergleich zu den anderen Varianten des Pyoderma gangrenosum zeigt dieser Subtyp in der Regel ein gutes Ansprechen auf weniger aggressive Behandlungen, und zugrundeliegende systemische Erkrankungen sind seltener assoziiert. Der vegetative Subtyp gilt als die gutartigste und seltenste klinische Variante des Pyoderma gangrenosum."},"es":{"name":"Pioderma gangrenoso vegetante","synonyms":["Pioderma gangrenoso granulomatoso"],"definition":"Es un subtipo poco frecuente de pioderma gangrenoso caracterizado por una placa ulcerada, eritematosa y aislada que carece del borde violáceo típico del pioderma gangrenoso clásico y que suele afectar a individuos sanos. Histológicamente, la lesión presenta una capa central que contiene inflamación neutrofílica, rodeada por una empalizada de histiocitos con un infiltrado linfocítico en el borde. En comparación con las otras variantes de pioderma gangrenoso, este subtipo, por lo general, muestra una buena respuesta a tratamientos menos agresivos y asocia trastornos sistémicos subyacentes con menor frecuencia. Está considerada como la variante clínica más benigna e inusual del pioderma gangrenoso."},"it":{"name":"Pioderma gangrenoso vegetativo","definition":"Si tratta di un sottotipo raro di pioderma gangrenoso, caratterizzato da una placca solitaria eritematosa ulcerata, che non presenta il bordo violaceo tipico del pioderma gangrenoso classico. Di solito colpisce i soggetti in buona salute. Dal punto di vista istologico, la lesione presenta uno strato centrale contenente una infiammazione neutrofila, circondato da istiociti disposti a palizzata e circondati da un infiltrato linfocitario. Rispetto alle altre forme del pioderma gangrenoso, questo sottotipo di solito risponde bene ai trattamenti meno aggressivi e si associa più raramente a malattie sistemiche preesistenti. Si tratta della variante clinica più benigna e più rara del pioderma gangrenoso."},"nl":{"name":"Vegetatieve pyoderma gangrenosum","synonyms":["Granulomateuze pyoderma gangrenosum"],"definition":"Een zeldzaam subtype van pyoderma gangrenosum dat gekarakteriseerd wordt door een solitaire, erythemateuze, ulceratieve plaque zonder de violette rand die doorgaans aanwezig is bij klassieke pyoderma gangrenosum, en dat meestal voor het overige gezonde individuen treft. Histologisch vertoont het letsel een centrale laag met neutrofiele inflammatie, omgeven door histiocyten die zijn omrand door een lymfocytair infiltraat. In vergelijking met andere varianten van pyoderma gangrenosum vertoont dit subtype doorgaans een goede respons op minder agressieve behandelingen en zijn minder vaak onderliggende systemische stoornissen geassocieerd. Het wordt beschouwd als de meest goedaardige en minst gangbare klinische variant van pyoderma gangrenosum."},"pt":{"name":"Pioderma gangrenoso vegetativo"},"pl":{"name":"Piodermia wegetatywna zgorzelinowa","synonyms":["Granulomatous pyoderma gangrenosum"]},"cs":{"name":"Vegetativní pyoderma gangrenosum","synonyms":["Granulomatózní pyoderma gangrenosum"]},"uk":{"name":"Вегетативна гангренозна піодермія","synonyms":["Гранульоматозна гангренозна піодермія"],"definition":"A rare subtype of pyoderma gangrenosum disease characterized by a solitary, erythematous, ulcerated plaque, which lacks the violaceous border typically present in classic pyoderma gangrenosum, usually affecting individuals who are otherwise healthy. Histologically, the lesion presents a central layer containing neutrophilic inflamation, surrounded by a palisade of histiocytes, which are rimmed by a lymphocytic infiltrate. In comparison with the other variants of pyoderma gangrenosum, this subtype usually shows a good response to less aggressive treatments and underlying systemic disorders are less frequently associated. It is considered the most benign and uncommon clinical variant of pyoderma gangrenosum."},"zh":{"name":"植物性坏疽性脓皮病","synonyms":["肉芽肿性坏疽性脓皮病"],"definition":"一种罕见的坏疽性脓皮病的亚型，以单发、红斑、溃疡性斑块为特征，缺乏典型坏疽性脓皮病中典型的紫色边界，通常影响健康的个体。在组织学上，病变的中心层含有中性粒细胞炎症，周围有一大片组织细胞，其边缘有淋巴细胞浸润。与其他变异性坏疽性脓皮病相比，这种亚型通常对不太积极的治疗有良好的反应，潜在的系统性疾病也较少发生。它被认为是最良性和最不常见的坏疽性脓皮病临床变种。"},"tr":{"name":"Vegetatif piyoderma gangrenozum","synonyms":["Granülomatöz 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":"824331367"},{"source":"MONDO","code":"0035238","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680158","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}