{"orpha":289347,"name":"Infective dermatitis associated with HTLV-1","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["IDH","Infective dermatitis associated with human T-lymphotropic virus type 1","Infective dermatitis associated with human T-lymphotropic virus type I"],"definition":"Infective dermatitis associated with HTLV-1 is a rare and severe chronic disease characterized by recurrent chronic eczema (with erythematous, scaly and crusted lesions) mainly affecting seborrheic areas (e.g. scalp, forehead, eyelids, paranasal and periauricular skin, neck, axillae, and groin), a generalized fine papular rash, chronic nasal discharge with crusting of the anterior nares, and non-virulent <i>Staphylococcus aureus</i> or beta-hemolytic <i>Streptococcus</i> infections, thought to be a result of HTLV-1-induced immunosuppression. Lymphadenopathy, anemia, mild to moderate pruritus and increased incidence of other infections (e.g. crusted scabies) have also been reported in some patients. Patients may subsequently develop other HTLV-1 associated conditions such as adult T-cell leukemia/lymphoma and tropical spastic paraparesis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-289347/","orphanet_url":"https://www.orpha.net/en/disease/detail/289347","names":{"fr":{"name":"Dermatite infectieuse associée au HTLV-1","synonyms":["Dermatite infectieuse associée au virus T-lymphotrope humain de type 1","Dermatite infectieuse associée au virus T-lymphotrope humain de type I","IDH"],"definition":"Maladie chronique rare et grave caractérisée par un eczéma chronique récurrent (à type de lésions érythémateuses, squameuses et croûteuses) touchant principalement les zones produisant du sébum (à savoir le cuir chevelu, le front, les paupières, la zone paranasale et périauriculaire, le cou, les aisselles et l'aine), une éruption papuleuse fine généralisée, un écoulement nasal chronique avec formation de croûtes dans la partie antérieure des narines et des infections non virulentes à Staphylocoque doré ou à streptocoques bêta-hémolytiques, probablement dues à l'immunosuppression induite par le HTLV-1. Une lymphadénopathie, une anémie, un prurit léger à modéré et une incidence accrue d'autres infections (par exemple, la gale croûteuse) ont également été rapportés chez certains patients. Les patients peuvent ensuite développer d'autres maladies associées au HTLV-1, telles que la leucémie/le lymphome T de l'adulte et la paraparésie spastique tropicale."},"de":{"name":"Infektiöse Dermatitis mit assoziiertem HTLV-1","synonyms":["IDH","Infektiöse Dermatitis mit assoziierter humaner T-lymphotroper Virus 1-Infektion"]},"es":{"name":"Dermatitis infecciosa asociada al HTLV-1","synonyms":["Dermatitis infecciosa asociada al virus linfotrópico de células T humanas tipo 1","Dermatitis infecciosa asociada al virus linfotrópico de células T humanas tipo I","IDH"],"definition":"Es una enfermedad grave, poco frecuente y crónica caracterizada por un eccema crónico recurrente (lesiones eritematosas, descamativas y costrosas) que afecta principalmente las áreas seborreicas (p. ej., cuero cabelludo, frente, párpados, región paranasal y preauricular, cuello, axilas e ingles), una erupción papular fina generalizada, exudación nasal crónica con formación de costras en las narinas e infecciones por <i>Staphylococcus aureus</i> o por <i>Streptococcus </i> beta-hemolítico, considerada como una consecuencia de la inmunosupresión inducida por HTLV-1. En algunos pacientes, también se han descrito poliadenopatías, anemia, prurito de intensidad variable y una mayor incidencia de otros procesos infecciosos (p. ej., sarna costrosa). Los individuos afectos pueden desarrollar posteriormente otras procesos asociados a HTLV-1, como un linfoma / leucemia de células T del adulto y una paraparesia espástica tropical."},"it":{"name":"Dermatite infettiva associata a HTLV-1","synonyms":["Dermatite infettiva associata a virus T-linfotropico umano tipo 1","Dermatite infettiva associata a virus T-linfotropico umano tipo I","IDH"],"definition":"La dermatite infettiva associata a HTLV-1 è una rara e grave malattia cronica, caratterizzata da eczema cronico ricorrente (con lesioni eritematose, squamose, e crostose) che interessa principalmente le aree seborroiche (cuoio capelluto, fronte, palpebre, cute paranasale e periauricolare, collo, ascelle, e inguine), un'eruzione generalizzata di papule, rinorrea cronica con formazione di croste sulla porzione anteriore delle narici ed infezioni non virulente da <i>Staphylococcus aureus</i> o beta-emolitiche da <i>Streptococcus</i>, che si ritiene siano la conseguenza di un'immunosoppressione indotta da HTLV-1. In alcuni pazienti, inoltre, si osservano linfadenopatia, anemia, prurito lieve-moderato ed un aumento dell'incidenza di altre infezioni (ad esempio, la scabbia crostosa). In seguito, i pazienti possono sviluppare altre malattie correlate a HTLV-1, come la leucemia/linfoma T dell'adulto e la paraparesi spastica tropicale (si vedano questi termini)."},"nl":{"name":"Infectieuze dermatitis geassocieerd met HTLV-1","synonyms":["IDH","Infectieuze dermatitis geassocieerd met humaan T-lymfotroop virus type 1","Infectieuze dermatitis geassocieerd met humaan T-lymfotroop virus type I"],"definition":"Infectieuze dermatitis geassocieerd met HTLV-1 is een zeldzame en ernstige chronische ziekte die gekarakteriseerd wordt door terugkerend chronisch eczeem (met erythemateuze, schilferende en crusteuze laesies) van voornamelijk de seborroïsche gebieden (e.g. hoofdhuid, voorhoofd, oogleden, paranasale en peri-auriculaire huid, hals/nek, oksels, en kruis), een gegeneraliseerde fijne papulaire huiduitslag, chronische neusafscheiding met korstvorming aan de neusgaten, en infecties met niet-virulente <i>Staphylococcus aureus</i> of bèta-hemolytische <i>Streptococcus</i>, vermoedelijk als gevolg van HTLV-1-geïnduceerde immuunsuppressie. Lymfadenopathie, anemie, milde tot matige pruritus en verhoogde incidentie van andere infecties (e.g. scabies crustosa (Noorse schurft)) werden ook reeds gerapporteerd bij sommige patiënten. Patiënten kunnen vervolgens andere HTLV-1-geassocieerde aandoeningen ontwikkelen, zoals volwassen T-celleukemie/lymfoom en tropische spastische paraparese (zie deze termen)."},"pt":{"name":"Dermatite infeciosa associada a HTLV-1","synonyms":["Dermatite infeciosa associada a vírus linfotrópico-T humano tipo 1","Dermatite infeciosa associada a vírus linfotrópico-T humano tipo I","IDH"]},"pl":{"name":"Zakaźne zapalenie skóry związane z HTLV-1","synonyms":["IDH","Zakaźne zapalenie skóry związane z ludzkim wirusem T-limfotropowym typu 1","Zakaźne zapalenie skóry związane z ludzkim wirusem T-limfotropowym typu I"]},"cs":{"name":"Infekční dermatitida asociovaná s HTLV-1","synonyms":["IDH","Infekční dermatitida asociovaná s lidským T-lymfotropickým virem typu 1","Infekční dermatitida asociovaná s lidským T-lymfotropickým virem typu I"]},"uk":{"name":"Інфекційний дерматит, асоційований з HTLV-1","synonyms":["IDH","Інфекційний дерматит, асоційований з Т-лімфотропним вірусом людини типу 1","Інфекційний дерматит, асоційований з Т-лімфотропним вірусом людини типу I"],"definition":"Infective dermatitis associated with HTLV-1 is a rare and severe chronic disease characterized by recurrent chronic eczema (with erythematous, scaly and crusted lesions) mainly affecting seborrheic areas (e.g. scalp, forehead, eyelids, paranasal and periauricular skin, neck, axillae, and groin), a generalized fine papular rash, chronic nasal discharge with crusting of the anterior nares, and non-virulent <i>Staphylococcus aureus</i> or beta-hemolytic <i>Streptococcus</i> infections, thought to be a result of HTLV-1-induced immunosuppression. Lymphadenopathy, anemia, mild to moderate pruritus and increased incidence of other infections (e.g. crusted scabies) have also been reported in some patients. Patients may subsequently develop other HTLV-1 associated conditions such as adult T-cell leukemia/lymphoma and tropical spastic paraparesis (see these terms)."},"zh":{"name":"与HTLV-1基因相关的感染性皮炎","synonyms":["人T淋巴细胞病毒I型相关性感染性皮炎"],"definition":"与HTLV-1相关感染性皮炎是一种罕见且严重的慢性疾病，其特征是复发性慢性湿疹（伴有红斑、鳞屑和结痂），主要影响脂溢区域（如头皮、额头、眼睑、副鼻腔和耳周皮肤、颈部、腋窝和腹股沟），全身弥漫性细丘疹，慢性流涕伴鼻前孔结痂，非毒性金黄色葡萄球菌或β溶血性链球菌感染，被认为是HTLV-1诱导的免疫抑制的结果。淋巴结肿大、贫血、轻度至中度瘙痒和其他感染（如结痂性疥疮）的发病率增加也在一些患者中有报道。患者随后可能出现其他HTLV-1相关疾病，如成人T细胞性白血病/淋巴瘤和热带痉挛性轻截瘫（见这些术语）。"},"tr":{"name":"HTLV-1-ilişkili enfektif dermatit","synonyms":["IDH","İnsan T-lenfotropik virüs tip 1 ilişkili infektif dermatit","İnsan T-lenfotropik virüs tip I ile seyreden infektif dermatit"]}},"xrefs":[{"source":"ICD-10","code":"L30.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EA88.00","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1924962677"},{"source":"MONDO","code":"0017326","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4274291","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":90077,"name":"Other acquired skin disease","classifications":[187]},{"orpha":163585,"name":"Rare viral disease","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}