{"orpha":451602,"name":"Primary cutaneous plasmacytosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare acquired skin disease characterized by benign proliferation of mature plasma cells with a typical triad of cutaneous lesions, polyclonal hypergammaglobulinemia, and superficial lymphadenopathy, without an apparent underlying cause. The skin lesions consist of multiple round-to-oval, red-to-dark-brown macules, papules, and plaques most commonly found on the trunk, but also the face, neck, and axillae.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-451602/","orphanet_url":"https://www.orpha.net/en/disease/detail/451602","names":{"fr":{"name":"Plasmocytose cutanée primitive","definition":"Maladie dermatologique acquise rare caractérisée par une prolifération bénigne de plasmocytes matures associée à une triade typique de lésions cutanées, à une hypergammaglobulinémie polyclonale et à une lymphadénopathie superficielle, en l'absence de toute cause sous-jacente apparente. Les lésions cutanées consistent en de multiples macules, papules et plaques de forme ronde à ovale et de couleur rouge à brun foncé, le plus souvent observées sur le tronc, mais aussi sur le visage, le cou et sous les aisselles."},"de":{"name":"Primär kutane Plasmazytose","definition":"Eine seltene erworbene Hauterkrankung, die durch eine gutartige Proliferation reifer Plasmazellen mit einer typischen Trias aus Hautläsionen, polyklonaler Hypergammaglobulinämie und oberflächlicher Lymphadenopathie gekennzeichnet ist, ohne dass eine erkennbare Ursache vorliegt. Die Hautläsionen bestehen aus multiplen runden bis ovalen, rot- bis dunkelbraunen Makeln, Papeln und Plaques, die am häufigsten am Rumpf, aber auch im Gesicht, am Hals und in den Achselhöhlen auftreten."},"es":{"name":"Plasmocitosis cutánea primaria","definition":"Es una enfermedad cutánea adquirida poco frecuente caracterizada por la proliferación benigna de células plasmáticas maduras junto con una tríada típica de lesiones cutáneas, hipergammaglobulinemia policlonal y linfadenopatía superficial, sin una causa subyacente aparente. Las lesiones cutáneas consisten en múltiples máculas, pápulas y placas redondeadas u ovaladas, de color rojo a marrón oscuro, que se desarrollan con mayor frecuencia en el tronco, aunque también en la cara, el cuello y las axilas."},"it":{"name":"Plasmocitosi cutanea primitiva","definition":"Si tratta di una malattia acquisita rara della cute, caratterizzata dalla proliferazione benigna di plasmacellule mature, con la tipica triade lesioni cutanee, ipergammaglobulinemia policlonale e linfoadenopatia superficiale. L'eziologia non è nota. Le lesioni cutanee sono costituite da macule, papule e placche multiple di forma arrotondata-ovale, di colore rosso-marrone scuro, in genere localizzate sul tronco, sul viso, sul collo e sulle ascelle."},"nl":{"name":"Primaire cutane plasmocytose","definition":"Een zeldzame verworven aandoening van huid, gekarakteriseerd door goedaardige proliferatie van rijpe plasmacellen en een typische triade van huidlaesies, polyklonale hypergammaglobulinemie, en oppervlakkige lymfadenopathie, zonder een aanwijsbare onderliggende oorzaak. De huidlaesies bestaan uit meerdere ronde tot ovale, rode tot donkerbruine maculae, papels en plaques die doorgaans worden aangetroffen op de romp, maar ook het gelaat, de hals en de oksels."},"pt":{"name":"Plasmocitose cutânea primária"},"pl":{"name":"Pierwotna plazmacytoza skórna"},"cs":{"name":"Primární kožní plazmocytóza"},"uk":{"name":"Первинний шкірний плазмоцитоз","definition":"A rare acquired skin disease characterized by benign proliferation of mature plasma cells with a typical triad of cutaneous lesions, polyclonal hypergammaglobulinemia, and superficial lymphadenopathy, without an apparent underlying cause. The skin lesions consist of multiple round-to-oval, red-to-dark-brown macules, papules, and plaques most commonly found on the trunk, but also the face, neck, and axillae."},"zh":{"name":"原发性皮肤浆细胞增多症"},"tr":{"name":"Primer kutanöz plazmasitoz"}},"xrefs":[{"source":"ICD-10","code":"L98.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EK91.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1669369613"},{"source":"MONDO","code":"0018679","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4736227","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":284264,"name":"IgG4-related disease","classifications":[196]},{"orpha":90077,"name":"Other acquired skin disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}