{"orpha":397587,"name":"Deep dermatophytosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Disseminated granulomatous dermatophytosis"],"definition":"A rare mycosis characterized by severe, potentially life-threatening dermal and subcutaneous tissue invasion by dermatophytes. Dissemination to lymph nodes is frequent, but the infection may also occasionally spread to the central nervous system. Cutaneous signs and symptoms include erythema, desquamation, itching, nodules, plaques, or ulceration. The majority of deep dermatophytoses develop in immunocompromised patients.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-397587/","orphanet_url":"https://www.orpha.net/en/disease/detail/397587","names":{"fr":{"name":"Dermatophytose profonde","synonyms":["Dermatophytie granulomateuse à foyers disséminés","Maladie dermatophytique"],"definition":"Mycose rare caractérisée par une infection sévère et potentiellement létale des tissus dermiques et sous-cutanés par des dermatophytes. La dissémination vers les ganglions lymphatiques est fréquente, mais l'infection peut aussi se propager occasionnellement vers le système nerveux central. Les signes cliniques et symptômes cutanés incluent l'érythème, la desquamation, les démangeaisons, les nodules, les plaques ou les ulcérations. La majorité des dermatophytoses sévères se développent chez les patients immunodéprimés."},"de":{"name":"Tiefe Dermatophytose","synonyms":["Disseminierte granulomatöse Dermatophytose"],"definition":"Eine seltene Mykose, die durch eine schwere, potenziell lebensbedrohliche Invasion von Dermatophyten in die Haut und das Unterhautgewebe gekennzeichnet ist. Häufig kommt es zu einer Streuung in die Lymphknoten, gelegentlich kann die Infektion auch auf das Zentralnervensystem übergreifen. Zu den Hauterscheinungen gehören Erythem, Schuppung, Juckreiz, Knötchen, Plaques oder Ulzerationen. Die meisten tiefen Dermatophytosen entwickeln sich bei immungeschwächten Patienten."},"es":{"name":"Dermatofitosis profunda","synonyms":["Dermatofitosis granulomatosa diseminada"],"definition":"Es una micosis poco frecuente caracterizada por una invasión grave y potencialmente mortal del tejido dérmico y subcutáneo por dermatofitos. La diseminación a los ganglios linfáticos es frecuente, aunque la infección también puede propagarse ocasionalmente al sistema nervioso central. Los signos y síntomas cutáneos incluyen eritema, descamación, prurito, nódulos, placas o úlceras. La mayoría de las dermatofitosis profundas se desarrollan en pacientes inmunodeprimidos."},"it":{"name":"Dermatofitosi profonda","synonyms":["Dermatofitosi granulomatosa disseminata"],"definition":"Si tratta di una micosi rara, caratterizzata da un'invasione grave e potenzialmente letale del derma e del tessuto sottocutaneo da parte dei dermatofiti. Spesso si osserva una disseminazione verso i linfonodi e, occasionalmente, a livello del sistema nervoso centrale. I sintomi cutanei comprendono l'eritema, la desquamazione, il prurito, i noduli, le placche e le ulcere. La malattia colpisce per lo più i pazienti immunocompromessi."},"nl":{"name":"Diepe dermatofytose","synonyms":["Verspreide granulomateuze dermatofytose"],"definition":"Een zeldzame mycose, gekarakteriseerd door ernstige, potentieel levensbedreigende invasie van dermaal en subcutaan weefsel door dermatofyten. Disseminatie naar lymfeklieren komt vaak voor, maar occasioneel kan de infectie ook verspreiden naar centraal zenuwstelsel. Cutane tekenen en symptomen zijn onder meer erytheem, desquamatie, jeuk, noduli, plaques, en ulceratie. De meeste gevallen van diepe dermatofytose komen voor bij immuungecompromitteerde patiënten."},"pt":{"name":"Dermatofitose profunda"},"pl":{"name":"Dermatofitoza głęboka","synonyms":["Rozsiana dermatofitoza ziarniniakowata"]},"cs":{"name":"Hluboká dermatofytóza","synonyms":["Diseminovaná granulomatózní dermatofytóza"]},"uk":{"name":"Глибокий дерматофітоз","synonyms":["Дисемінована гранульоматозна дерматофітія"],"definition":"A rare mycosis characterized by severe, potentially life-threatening dermal and subcutaneous tissue invasion by dermatophytes. Dissemination to lymph nodes is frequent, but the infection may also occasionally spread to the central nervous system. Cutaneous signs and symptoms include erythema, desquamation, itching, nodules, plaques, or ulceration. The majority of deep dermatophytoses develop in immunocompromised patients."},"zh":{"name":"深部皮肤癣菌病","synonyms":["播散肉芽肿性皮肤霉菌病"],"definition":"本病是一种罕见的霉菌病，其特征是皮肤癣菌对皮肤和皮下组织的侵袭严重，可危及生命。转移到淋巴结的概率很高，感染偶尔也能扩散至中枢神经系统。皮肤体征和症状包括红斑、脱皮、瘙痒、结节、斑块或溃疡。大多数深部皮肤癣病发生在免疫功能低下的患者身上。"},"tr":{"name":"Derin dermatofitoz","synonyms":["Yaygın granülomatöz dermatofitoz"]}},"xrefs":[{"source":"ICD-10","code":"B35.8","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"1F28.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0021660","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1395264","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":["Adult","Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}