{"orpha":86797,"name":"Atypical lichen myxedematosus","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Intermediate lichen myxedematosus"],"definition":"An intermediate form of lichen myxedematosus (LM) (a form of mucin dermal deposit) which does not meet the criteria for either scleromyxedema or the localized form. Three clinical subtypes have been described and include scleromyxedema without monoclonal gammopathy; localized forms with monoclonal gammopathy and/or systemic symptoms; localized forms with mixed features of the 5 subtypes of localized LM (discrete form, acral persistent papular mucinosis, self-healing papular mucinosis, papular mucinosis of infancy, and a pure nodular form). The course of atypical LM is unpredictable because only a few cases have been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86797/","orphanet_url":"https://www.orpha.net/en/disease/detail/86797","names":{"fr":{"name":"Lichen myxoedémateux atypique","synonyms":["Lichen myxoedémateux intermédiaire"],"definition":"Le lichen myxoedémateux atypique est une forme intermédiaire de lichen myxoedémateux (LM) (une forme de mucinose dermique) ne remplissant pas les mêmes critères que le scléromyxoedème ou la forme localisée. Trois sous-types cliniques ont été décrits, comprenant le scléromyxoedème sans gammopathie monoclonale ; les formes localisées avec gammopathie monoclonale et/ou symptômes systémiques ; ainsi que les formes localisées à présentation mixte (forme discrète, mucinose papuleuse acrale persistante, mucinose cutanée juvénile spontanément régressive, mucinose cutanée infantile, et forme nodulaire pure). L'évolution du LM atypique est imprévisible car seuls quelques cas ont été rapportés à ce jour."},"de":{"name":"Lichen myxoedematosus, atypischer","synonyms":["Intermediärer Lichen myxödematosus"]},"es":{"name":"Liquen mixedematoso atípico","synonyms":["Liquen mixedematoso intermedio"],"definition":"Es una forma intermedia de liquen mixedematoso (LM) (una forma de depósito dérmico de mucina) que no cumple los criterios del escleromixedema o de la forma localizada. Se han descrito tres subtipos clínicos quee incluyen el escleromixedema sin gammapatía monoclonal; las formas localizadas con gammapatía monoclonal y/o síntomas sistémicos; formas localizadas con características mixtas de los 5 subtipos de LM localizado (forma discreta, mucinosis papular acral persistente, mucinosis papular de resolución espontánea, mucinosis papular de la infancia y una forma nodular pura). El curso del LM atípico es impredecible dado que sólo se han descrito unos pocos casos en la literatura médica."},"it":{"name":"Lichen mixedematoso atipico","synonyms":["Lichen mixedematoso intermedio"]},"nl":{"name":"Atypische lichen myxoedematosus","synonyms":["Intermediaire lichen myxoedematosus"],"definition":"Een intermediaire vorm van lichen myxoedematosus (LM) (een vorm van mucine-afzetting in huid) die niet voldoet aan de criteria voor scleromyxoedeem of voor de gelokaliseerde vorm. Er werden drie klinische subtypes beschreven, zijnde scleromyxoedeem zonder monoklonale gammopathie; gelokaliseerde vormen met monoklonale gammopathie en/of systemische symptomen; gelokaliseerde vormen met gemengde kenmerken van de 5 subtypes van gelokaliseerde LM (discrete vorm, acrale persistente papulaire mucinose, zelfhelende papulaire mucinose, papulaire mucinose in de zuigelingentijd, en een zuiver nodulaire vorm). Het verloop van atypische LM is onvoorspelbaar aangezien er nog maar weinig gevallen werden gerapporteerd."},"pt":{"name":"Líquen mixedematoso, formas atípicas"},"pl":{"name":"Atypowy liszaj śluzowaty","synonyms":["Liszaj śluzowaty średniozaawanowany"]},"cs":{"name":"Atypický lichen myxedematosus","synonyms":["Středně pokročilý lichen myxedematosus"]},"uk":{"name":"Атиповий мікседематозний ліхен","synonyms":["Проміжний лишай мікседематозний"],"definition":"An intermediate form of lichen myxedematosus (LM) (a form of mucin dermal deposit) which does not meet the criteria for either scleromyxedema or the localized form. Three clinical subtypes have been described and include scleromyxedema without monoclonal gammopathy; localized forms with monoclonal gammopathy and/or systemic symptoms; localized forms with mixed features of the 5 subtypes of localized LM (discrete form, acral persistent papular mucinosis, self-healing papular mucinosis, papular mucinosis of infancy, and a pure nodular form). The course of atypical LM is unpredictable because only a few cases have been reported."},"zh":{"name":"非典型粘液水肿性苔癣","synonyms":["中间型黏液水肿性苔藓"]},"tr":{"name":"Atipik liken miksödemöz","synonyms":["Orta dereceli liken miksödemöz"]}},"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},{"source":"MONDO","code":"0019447","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4510874","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":90398,"name":"Localized lichen myxedematosus with mixed features of different subtypes","type":"Clinical subtype"},{"orpha":90399,"name":"Localized lichen myxedematosus with monoclonal gammopathy or systemic symptoms","type":"Clinical subtype"},{"orpha":90400,"name":"Scleromyxedema without monoclonal gammopathy","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":20.0,"area":"Worldwide","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}