{"orpha":364039,"name":"Hydroa vacciniforme-like lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Angiocentric cutaneous T-cell lymphoma of childhood","HVLL","Hydroa-like cutaneous T-cell lymphoma"],"definition":"A very rare Epstein-Barr virus-associated lymphoproliferative disorder characterized by a chronic, recurrent, vesiculopapular rash, which subsequently ulcerates and scars, located mainly on sun-exposed areas and which is associated with systemic manifestations, such as fever, weight loss, asthenia, facial edema, arthralgia, lymphadenopathy, hepatosplenomegaly and/or increased liver enzymes. Hypersensitivity to mosquito bites has been associated and an increased risk of developing systemic lymphoma has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-364039/","orphanet_url":"https://www.orpha.net/en/disease/detail/364039","names":{"fr":{"name":"Lymphome à type d'hydroa vacciniforme","definition":"Un trouble lymphoprolifératif malin très rare, associé au virus d'Epstein-Barr. Il se caractérise par une éruption vésiculopapulaire récurrente chronique avec ulcération et formation de cicatrices, située principalement au niveau des zones du corps exposées au soleil. Des manifestations systémiques sont associées, telles qu'une fièvre, une perte de poids, une asthénie, un oedème du visage, une arthralgie, une lymphadénopathie, une hépatosplénomégalie et/ou des taux élevés d'enzymes hépatiques. Une hypersensibilité aux piqûres de moustique a été associée, ainsi qu'un risque accru de développer un lymphome systémique."},"de":{"name":"Hydroa vacciniforme-ähnliche lymphoproliferative Erkrankung","synonyms":["Hydroa vacciniforme-artige lymphoproliferative Krankheit","Hydroa-artiges kutanes T-Zell-Lymphom","Angiozentrisches kutanes T-Zell-Lymphom der Kindheit","HV-LPD"],"definition":"Eine sehr seltene Epstein-Barr-Virus-assoziierte lymphoproliferative Erkrankung, die durch einen chronisch rezidivierenden vesikulopapulösen Hautausschlag gekennzeichnet ist, der später ulzeriert und vernarbt und hauptsächlich an sonnenexponierten Stellen auftritt. Er ist verbunden mit systemischen Manifestationen wie Fieber, Gewichtsverlust, Asthenie, Gesichtsödem, Arthralgie, Lymphadenopathie, Hepatosplenomegalie und/oder erhöhten Leberenzymen. Eine Überempfindlichkeit gegen Mückenstiche wurde assoziiert und es wurde über ein erhöhtes Risiko für die Entwicklung eines systemischen Lymphoms berichtet."},"es":{"name":"Linfoma similar a hidroa vacciniforme","synonyms":["HVLL","Linfoma cutáneo de células T angiocéntrico de la infancia","Linfoma cutáneo de células T similar a hidroa","Linfoma de hidroa vacciniforme-like"],"definition":"Es un trastorno linfoproliferativo muy poco frecuente asociado al virus de Epstein-Barr que se caracteriza por una erupción vesiculopapular crónica y recurrente, que posteriormente se ulcera y cicatriza, localizada principalmente en áreas expuestas al sol y que se asocia con manifestaciones sistémicas, como fiebre, pérdida de peso, astenia, edema facial, artralgia, linfadenopatía, hepatoesplenomegalia y/o aumento de las enzimas hepáticas. Se ha asociado a hipersensibilidad a la picadura de mosquitos y se ha notificado un mayor riesgo de desarrollar linfoma sistémico."},"it":{"name":"Linfoma idroa vacciniforme-simile","definition":"Il linfoma simil-idroa vacciniforme è una malattia linfoproliferativa maligna molto rara associata al virus di Epstein-Barr. È caratterizzata da eruzioni vesciculo-papulari ricorrenti croniche, seguite dalla formazione di ulcere e cicatrici, localizzate soprattutto nelle zone esposte al sole. I pazienti presentano sintomi sistemici come febbre, perdita di peso, astenia, edema del viso, artralgia, linfoadenopatia, epatosplenomegalia e/o aumento dei valori degli enzimi epatici. Si associa ad ipersensibilità alle punture di zanzara e ad un aumento del rischio di linfoma sistemico."},"nl":{"name":"Hydroa vacciniforme-achtig lymfoom","synonyms":["Angiocentrisch cutaan T-cellymfoom in de kindertijd","HVLL","Hydroa-achtig cutaan T-cellymfoom"],"definition":"Een zeer zeldzame, Epstein-Barrvirus-geassocieerde, maligne, lymfoproliferatieve aandoening, gekarakteriseerd door een chronische, terugkerende, vesiculopapulaire huiduitslag die vervolgens verzweert en littekens vormt, voornamelijk op plaatsen die worden blootgesteld aan de zon, en die is geassocieerd met systemische manifestaties, zoals koorts, gewichtsverlies, asthenie, gezichtsoedeem, artralgie, lymfadenopathie, hepatosplenomegalie en/of verhoogde leverenzymen. Hypersensitiviteit voor muggenbeten werd geassocieerd en een verhoogd risico op de ontwikkeling van systemisch lymfoom werd gerapporteerd."},"pt":{"name":"Linfoma semelhante a Hidroa vaciniforme"},"pl":{"name":"Chłoniak typu opryszczki ospówkowatej","synonyms":["HVLL"]},"cs":{"name":"Lymfom \"Hydroa vacciniforme-like","synonyms":["Angiocentric cutaneous T-cell lymphoma of childhood","HVLL","Hydroa-like cutaneous T-cell lymphoma"]},"uk":{"name":"Hydroa vacciniforme-подібна лімфома","synonyms":["Ангіоцентрична шкірна Т-клітинна лімфома дитячого віку","HVLL","Гідроа-подібна шкірна Т-клітинна лімфома"],"definition":"A very rare Epstein-Barr virus-associated lymphoproliferative disorder characterized by a chronic, recurrent, vesiculopapular rash, which subsequently ulcerates and scars, located mainly on sun-exposed areas and which is associated with systemic manifestations, such as fever, weight loss, asthenia, facial edema, arthralgia, lymphadenopathy, hepatosplenomegaly and/or increased liver enzymes. Hypersensitivity to mosquito bites has been associated and an increased risk of developing systemic lymphoma has been reported."},"zh":{"name":"牛痘水疱样淋巴瘤","synonyms":["儿童血管中心皮肤T细胞淋巴瘤"],"definition":"本病是一种非常罕见的与EB病毒相关的淋巴增生性疾病，其特征为慢性、复发性、水泡性丘疹，随后出现溃疡和疤痕，主要位于阳光照射区的，并与全身症状有关，如发烧、体重减轻、虚弱、面部水肿、关节痛、淋巴结病变、肝脾肿大和/或肝酶升高。被蚊子叮咬后的高过敏反应与本病相关。有报道表明患病后全身性淋巴瘤的风险增加。"},"tr":{"name":"Hydroa vacciniforme benzeri lenfoma","synonyms":["HVLL","Hidroa-benzeri kutanöz T-hücreli lenfoma","Çocukluk çağı anjiyosentrik kutanöz T-hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C84.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"42531582"},{"source":"MONDO","code":"0018224","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1708397","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]},{"orpha":171918,"name":"T-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Childhood"],"prevalence":[],"phenotypes":null}