{"orpha":826,"name":"Sporotrichosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Sporotrichosis is an infection caused by the dimorphic fungus <i>Sporothrix schenckii</i>, generally occurring by traumatic inoculation of fungus from contaminated soil, plants, and organic matter, that has a highly variable disease spectrum but that usually presents as a subcutaneous mycosis with a single sporotrichotic chancre that may ulcerate and can then progress to lymphocutaneous (most common form; sporotrichotic chancre at inoculation site and a string of similar nodules along the proximal lymphatics), fixed cutaneous (localized asymptomatic, erythematous, papules at the inoculation site), or multifocal or disseminated cutaneous (rare form, with 3 or more lesions involving 2 different anatomical sites) forms. Pulmonary sporotrichosis occurs following inhalation of fungus and manifests as chronic pneumonitis while extracutaneous or systemic sporotrichosis (with osteoarticular, pulmonary, and central nervous system/meningeal disease) has also been reported, usually occurring in the setting of immunosuppression.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-826/","orphanet_url":"https://www.orpha.net/en/disease/detail/826","names":{"fr":{"name":"Sporotrichose","definition":"Infection causée par le champignon dimorphique <i>Sporothrix schenckii</i>, survenant généralement par inoculation du champignon à la suite d'un traumatisme cutané, à partir de sols, de végétaux et de matières organiques contaminés. La maladie couvre un spectre très variable, mais se présente généralement sous la forme d'une mycose sous-cutanée avec un seul chancre sporotrichosique qui peut s'ulcérer et évoluer vers une forme lymphocutanée (forme la plus courante, chancre sporotrichotique au site d'inoculation et série de nodules le long des vaisseaux lymphatiques proximaux), une forme cutanée (papules érythémateuses asymptomatiques localisées au site d'inoculation), ou une forme cutanée multifocale ou disséminée (forme rare, avec trois lésions ou plus impliquant deux parties anatomiques différentes). La sporotrichose pulmonaire survient à la suite de l'inhalation du champignon et se manifeste par une pneumopathie chronique, tandis que la sporotrichose extracutanée ou systémique (avec atteinte ostéo-articulaire, pulmonaire et du système nerveux central/méningée) a généralement été décrite dans un contexte d'immunosuppression."},"de":{"name":"Sporotrichose","definition":"Die Sporotrichose ist eine Infektion, die durch den dimorphen Pilz <i>Sporothrix schenckii</i> verursacht wird. Die Infektion erfolgt im Allgemeinen durch traumatische Inokulation von Pilzsporen aus kontaminierten Böden, Pflanzen und anderen organischen Substanzen. Die Sporotrichose weist ein sehr variables Krankheitsbild auf, das sich jedoch typischerweise als subkutan Mykose mit einem einzelnen sporotrichotischen Schanker manifestiert. Dieser kann ulzerieren und sich zu einer lymphokutanen Form (häufigste Form; sporotrichotischer Schanker an der Inokulationsstelle und eine Reihe ähnlicher Knötchen entlang der proximalen Lymphgefäße), einer fixiert kutanen Form (lokalisiert asymptomatisch, erythematös, Papeln an der Inokulationsstelle) oder einer multifokale bzw. disseminierte kutanen Formen (seltene Form, mit drei oder mehr Läsionen an zwei verschiedenen anatomischen Stellen) entwickeln. Eine pulmonale Sporotrichose tritt nach Inhalation von Pilzen auf und manifestiert sich als chronische Pneumonitis. Es wurde auch über extrakutane oder systemische Sporotrichosen (mit osteoartikuläre, Lungen- und Zentralnervensystem /meningealer Erkrankung) berichtet, die üblicherweise im Rahmen einer Immunsuppression auftreten."},"es":{"name":"Esporotricosis","definition":"Es una infección causada por el hongo dimórfico <i>Sporothrix schenckii</i>, que por lo general se produce por inoculación traumática del hongo presente en suelos, plantas y materia orgánica contaminados. La enfermedad abarca un espectro muy variable pero, por lo habitual, se presenta como una micosis subcutánea con un único chancro esporotricósico que puede ulcerarse y progresar a la forma linfocutánea (la forma más común; chancro esporotricósico en los puntos de inoculación y una serie de nódulos a lo largo de los vasos linfáticos proximales), a la forma cutánea (asintomática localizada, eritematosa, pápulas en el punto de inoculación), o a la forma cutánea multifocal o diseminada (forma rara, con 3 ó más lesiones que engloban dos puntos anatómicos diferentes). La esporotricosis pulmonar se produce tras la inhalación del hongo y se manifiesta como una neumonitis crónica, mientras que la esporotricosis extracutánea o sistémica (con enfermedad osteoarticular, pulmonar y del sistema nervioso central / meníngea) se ha descrito, por lo habitual, en situaciones de inmunosupresión."},"it":{"name":"Sporotricosi","definition":"La sporotricosi è un'infezione causata da un fungo dimorfico, lo Sporothrix schenckii, che di solito insorge dopo inoculazione traumatica del fungo presente nei terreni, nelle piante e nei materiali organici contaminati. Il quadro clinico è molto variabile, ma di solito si evidenzia con una micosi sottocutanea associata a sifiloma primitivo sporotricotico isolato, che può ulcerarsi ed evolvere in diverse forme: linfocutanea (la più comune; con sifiloma primitivo sporotricotico nel sito di inoculazione ed una serie di noduli analoghi lungo il segmento prossimale dei vasi linfatici); cutanea circoscritta (con papule eritematose localizzate e asintomatiche nel sito di inoculazione); cutanea multifocale o disseminata (forma rara; con tre o più lesioni che interessano due siti anatomici diversi). La sporotricosi polmonare è causata dall'inalazione del fungo e si manifesta con polmonite cronica. È stata descritta anche una forma di sporotricosi extracutanea o sistemica (associata a patologia osteoarticolare, polmonare, e meningea/del sistema nervoso centrale), che di solito insorge in presenza di una condizione di immunosoppressione."},"nl":{"name":"Sporotrichose","definition":"Sporotrichose is een infectie die veroorzaakt wordt door de dimorfe fungus <i>Sporothrix schenckii</i>, doorgaans door traumatische inoculatie van de fungus uit gecontamineerde aarde, planten en organisch materiaal. Sporotrichose vertoont een zeer variabel ziektebeeld maar komt meestal voor als een subcutane mycose met één enkele sporotrichotische sjanker die kan verzweren, en kan dan evolueren naar lymfocutane (meest voorkomende vorm; sporotrichotische sjanker ter hoogte van de plaats van inoculatie en een rij soortgelijke noduli langsheen de proximale lymfevaten), gefixeerde cutane (gelokaliseerd asymptomatisch, erythemateus, papels ter hoogte van de plaats van inoculatie), of multifocale of verspreide cutane (zeldzame vorm, met 3 of meer laesies op minstens 2 verschillende anatomische plaatsen) vormen. Pulmonale sporotrichose komt voor na inademing van de fungus en manifesteert zich als chronische pneumonitis, terwijl extracutane of systemische sporotrichose (osteo-articulaire, pulmonale, en centraal zenuwstelsel/meningeale ziekte), die ook reeds werd gerapporteerd, meestal voorkomt in de context van immuunsuppressie."},"pt":{"name":"Esporotricose"},"pl":{"name":"Sporotrychoza"},"cs":{"name":"Sporotrichóza"},"uk":{"name":"Споротрихоз","definition":"Sporotrichosis is an infection caused by the dimorphic fungus <i>Sporothrix schenckii</i>, generally occurring by traumatic inoculation of fungus from contaminated soil, plants, and organic matter, that has a highly variable disease spectrum but that usually presents as a subcutaneous mycosis with a single sporotrichotic chancre that may ulcerate and can then progress to lymphocutaneous (most common form; sporotrichotic chancre at inoculation site and a string of similar nodules along the proximal lymphatics), fixed cutaneous (localized asymptomatic, erythematous, papules at the inoculation site), or multifocal or disseminated cutaneous (rare form, with 3 or more lesions involving 2 different anatomical sites) forms. Pulmonary sporotrichosis occurs following inhalation of fungus and manifests as chronic pneumonitis while extracutaneous or systemic sporotrichosis (with osteoarticular, pulmonary, and central nervous system/meningeal disease) has also been reported, usually occurring in the setting of immunosuppression."},"zh":{"name":"孢子丝菌病"},"tr":{"name":"Sporotrikoz"}},"xrefs":[{"source":"ICD-10","code":"B42.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B42.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B42.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B42.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B42.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1F2J","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"579570784"},{"source":"ICD-11","code":"1F2J.0","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"768312788"},{"source":"ICD-11","code":"1F2J.1","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1370512518"},{"source":"ICD-11","code":"1F2J.2","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1229440796"},{"source":"ICD-11","code":"1F2J.3","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"984175752"},{"source":"ICD-11","code":"1F2J.Y","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0005968","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D013174","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0038034","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7692","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041736","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":163591,"name":"Rare mycosis","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25526781[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":">1 / 1000","value":2500.0,"area":"Mexico","status":"Validated","source":"25526781[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":54.0,"area":"Peru","status":"Validated","source":"10619735[PMID]"}],"phenotypes":null}