{"orpha":86795,"name":"Localized lichen myxedematosus","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["Papular mucinosis"],"definition":"A group of skin diseases characterized by the development of papules, nodules and/or plaques with mucin deposits and a variable degree of fibrosis in the absence of thyroid disease. The group comprises five sub-forms: nodular lichen myxedematosus, discrete papular lichen myxedematosus, papular mucinosis of infancy, acral persistent papular mucinosis and self-healing papular mucinosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86795/","orphanet_url":"https://www.orpha.net/en/disease/detail/86795","names":{"fr":{"name":"Lichen myxoedémateux localisé","synonyms":["Mucinose papuleuse"],"definition":"Groupe de maladies cutanées caractérisées par le développement de papules, de nodules et/ou de plaques avec dépôt de mucines, et un degré variable de fibrose sans maladie thyroïdienne. Le groupe comprend cinq sous-types : le lichen myxoedémateux nodulaire, le lichen myxoedémateux discret, la mucinose cutanée infantile, la mucinose papuleuse acrale persistante et la mucinose cutanée juvénile spontanément régressive."},"de":{"name":"Lichen myxoedematosus, lokalisierter","synonyms":["Muzinose, papulöse"],"definition":"Eine Gruppe von Hautkrankheiten, die durch die Entwicklung von Papeln, Knötchen und/oder Plaques mit Mucinablagerungen und einem variablen Grad an Fibrose ohne Schilddrüsenerkrankung gekennzeichnet sind. Die Gruppe umfasst fünf Unterformen: nodulärer Lichen myxedematosus, diskreter papulöser Lichen myxedematosus, papulöse Mucinose im Säuglingsalter, persistierende akrale papulöse Mucinose und selbstheilende papulöse Mucinose."},"es":{"name":"Liquen mixedematoso localizado","synonyms":["Mucinosis papular"],"definition":"Es un grupo de enfermedades de la piel caracterizadas por el desarrollo de pápulas, nódulos y/o placas con depósitos de mucina y un grado variable de fibrosis en ausencia de una enfermedad tiroidea. El grupo incluye cinco sub-formas: liquen mixedematoso nodular, liquen mixedematoso papular discreto, mucinosis papular de la infancia, mucinosis papular acral persistente y mucinosis papular de resolución espontánea."},"it":{"name":"Lichen mixedematoso localizzato","synonyms":["Mucinosi papulosa"],"definition":"Il lichen mixedematoso localizzato comprende un gruppo di malattie cutanee caratterizzate dallo sviluppo di papule, noduli e/o placche con depositi di mucina e gradi variabili di fibrosi, in assenza di una tireopatia. Il gruppo comprende cinque forme: il lichen mixedematoso nodulare, il lichen mixedematoso papuloso discreto, la mucinosi papulosa dell'infanzia, la mucinosi papulosa persistente acrale e la mucinosi papulosa spontaneamente regressiva."},"nl":{"name":"Gelokaliseerde lichen myxoedematosus","synonyms":["Papulaire mucinose"],"definition":"Een groep van aandoeningen van huid, gekenmerkt door ontwikkeling van papels, noduli en/of plaques met deposities van mucine en variabele mate van fibrose in afwezigheid van aandoening van schildklier. De groep omvat vijf subtypes: nodulaire lichen myxedematosus, afzonderlijke papulaire lichen myxedematosus, papulaire mucinose in de zuigelingentijd, acrale persisterende papulaire mucinose, en zelfhelende papulaire mucinose."},"pt":{"name":"Líquen mixedematoso localizado"},"pl":{"name":"Liszaj śluzowaty ograniczony","synonyms":["Liszaj śluzowaty"]},"cs":{"name":"Lokalizovaný lichen myxedematosus","synonyms":["Papulární mucinóza"]},"uk":{"name":"Локалізований мікседематозний ліхен","synonyms":["Папульозний муциноз"],"definition":"A group of skin diseases characterized by the development of papules, nodules and/or plaques with mucin deposits and a variable degree of fibrosis in the absence of thyroid disease. The group comprises five sub-forms: nodular lichen myxedematosus, discrete papular lichen myxedematosus, papular mucinosis of infancy, acral persistent papular mucinosis and self-healing papular mucinosis."},"zh":{"name":"局限性粘液水肿性苔癣","synonyms":["丘疹性黏蛋白病"]},"tr":{"name":"Lokalize liken miksödemöz","synonyms":["Papüler müsinoz"]}},"xrefs":[{"source":"ICD-11","code":"EB90.11","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"15830032"},{"source":"MONDO","code":"0019446","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5575846","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7321","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":402007,"name":"Lichen myxedematosus","classifications":[187]}],"children":[{"orpha":90396,"name":"Acral persistent papular mucinosis","type":"Disease"},{"orpha":90394,"name":"Discrete papular lichen myxedematosus","type":"Disease"},{"orpha":90393,"name":"Nodular lichen myxedematosus","type":"Disease"},{"orpha":90395,"name":"Papular mucinosis of infancy","type":"Disease"},{"orpha":90397,"name":"Self-healing papular mucinosis","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}