{"orpha":90399,"name":"Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms is a form of atypical lichen myxedematosus, characterized by the appearance of several 2-4 mm erythematous waxy papules confined to a few sites that may be associated with either an immunoglobulin A (IgA) nephropathy in patients with acral persistent papular mucinosis; discrete papular lichen myxedematosus; a scleromyxedema-like involvement, with dysphagia, hoarseness, pulmonary involvement, and carpal tunnel syndrome; myositis without skin sclerosis; or paraproteinemia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90399/","orphanet_url":"https://www.orpha.net/en/disease/detail/90399","names":{"fr":{"name":"Lichen myxoedémateux localisé avec gammopathie monoclonale ou symptômes systémiques","definition":"Le lichen myxoedémateux localisé avec gammopathie monoclonale ou symptômes systémiques est une forme de lichen myxoedémateux atypique caractérisée par l'apparition de plusieurs papules érythémateuses d'allure cireuse de 2 à 4 mm confinées à quelques sites. Elle peut être associée à : une néphropathie à immunoglobuline A (IgA) chez des patients atteints de mucinose papuleuse acrale persistante ; à une mucinose cutanée discrète ; à une atteinte de type scléromyxoedème avec dysphagie, enrouement, atteinte pulmonaire et syndrome du canal carpien ; à une myosite sans sclérose cutanée ; ou à une paraprotéinémie."},"de":{"name":"Lichen myxoedematosus, lokalisierter, mit monoklonaler Gammopathie/oder systemischen Symptomen"},"es":{"name":"Liquen mixedematoso localizado con gammapatía monoclonal o síntomas sistémicos","definition":"Es una forma de liquen mixedematoso atípico caracterizado por la aparición de varias pápulas cerosas eritematosas de 2-4 mm confinadas a unos pocos sitios que pueden estar asociadas a nefropatía por inmunoglobulina A (IgA) en pacientes con mucinosis papular acral persistente; liquen mixedematoso papular discreto; una afectación tipo escleromixedema, con disfagia, ronquera, afectación pulmonar y síndrome del túnel carpiano; miositis sin esclerosis de la piel; o paraproteinemia."},"it":{"name":"Lichen mixedematoso localizzato con gammapatia monoclonale e/o sintomi sistemici"},"nl":{"name":"Gelokaliseerde lichen myxoedematosus met monoklonale gammopathie of systemische symptomen","definition":"Gelokaliseerde lichen myxoedematosus met monoklonale gammopathie of systemische symptomen is een vorm van atypische lichen myxoedematosus (zie deze term) die wordt gekarakteriseerd door de aanwezigheid van verscheidene 2-4 mm grote, erythemateuze, wasachtige papels die zijn beperkt tot enkele plaatsen en die kunnen geassocieerd zijn met ofwel een immunoglobuline A (IgA) nefropathie in patiënten met acrale persistente papulaire mucinose; of discrete papulaire lichen myxoedematosus (zie deze termen); of een scleromyxoedeem-achtige betrokkenheid, met dysfagie, heesheid, pulmonale betrokkenheid, en carpaal tunnelsyndroom; of myositis zonder huidsclerose; of paraproteïnemie."},"pt":{"name":"Líquen mixedematoso localizado com gamopatia monoclonal ou sintomas sistémicos"},"pl":{"name":"Zlokalizowany liszaj śluzowaty z gammopatią monoklonalną lub objawami ogólnymi"},"cs":{"name":"Lokalizovaný lichen myxedematosus s monoklonální gamapatií nebo systémovými symptomy"},"uk":{"name":"Локалізований мікседематозний ліхен з моноклональною гамопатією або системними симптомами","definition":"Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms is a form of atypical lichen myxedematosus (see this term), characterized by the appearance of several 2-4 mm erythematous waxy papules confined to a few sites that may be associated with either an immunoglobulin A (IgA) nephropathy in patients with acral persistent papular mucinosis; discrete papular lichen myxedematosus (see these terms); a scleromyxedema-like involvement, with dysphagia, hoarseness, pulmonary involvement, and carpal tunnel syndrome; myositis without skin sclerosis; or paraproteinemia."},"zh":{"name":"局限性粘液水肿性苔癣伴单克隆丙种球蛋白病或全身症状"},"tr":{"name":"Monoklonal gammopati veya sistemik semptomlar ile seyreden lokalize liken miksödematozus"}},"xrefs":[{"source":"ICD-10","code":"L98.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB90.11","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0019584","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681466","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":86797,"name":"Atypical lichen myxedematosus","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":5.0,"area":"Worldwide","status":"Validated","source":"22235972[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}