{"orpha":90400,"name":"Scleromyxedema without monoclonal gammopathy","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"Scleromyxedema without monoclonal gammopathy is a form of atypical lichen myxedematosus, characterized by a generalized sclerodermoid infiltration of skin studded with multiple, firm papules of 1-3 mm in diameter involving face (leonine appearance), trunk, and limbs, without monoclonal gammopathy. The involvement of the face can be missing and pruritus may be prominent.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90400/","orphanet_url":"https://www.orpha.net/en/disease/detail/90400","names":{"fr":{"name":"Scléromyxoedème sans gammopathie monoclonale","definition":"Le scléromyxoedème sans gammopathie monoclonale est une forme de lichen myxoedémateux atypique caractérisée par une infiltration sclérodermiforme généralisée parsemant la peau de multiples papules fermes de 1 à 3 mm de diamètre sur le visage (apparence léonine), le tronc et les membres, sans gammopathie monoclonale. Le visage n'est pas toujours atteint et un prurit important peut être présent."},"de":{"name":"Skleromyxödem ohne monoklonale Gammopathie"},"es":{"name":"Escleromixedema sin gammapatía monoclonal","definition":"Es una forma de liquen mixedematoso atípico caracterizado por una infiltración esclerodermoide generalizada de la piel salpicada de múltiples pápulas firmes de 1-3 mm de diámetro que afectan a la cara (aspecto leonino), al tronco y a las extremidades, sin gammapatía monoclonal. En algunos casos, la cara no se ve afectada y el prurito puede ser intenso."},"it":{"name":"Scleromixedema senza gammopatia monoclonale"},"nl":{"name":"Scleromyxoedeem zonder monoklonale gammopathie","definition":"Scleromyxoedeem zonder monoklonale gammopathie is een vorm van atypische lichen myxoedematosus (zie deze term) die wordt gekarakteriseerd door een gegeneraliseerde sclerodermoïde infiltratie van huid bezaaid met meerdere stevige papels met een diameter van 1-3 mm aan het gelaat (facies leonina of leeuwengezicht), de romp, en de ledematen, zonder monoklonale gammopathie. Het is mogelijk dat het gelaat niet getroffen is, en pruritus kan nadrukkelijk aanwezig zijn."},"pt":{"name":"Escleromixedema sem gamopatia monoclonal"},"pl":{"name":"Liszaj śluzowaty twardzinowy bez gammopatii monoklonalnej"},"cs":{"name":"Skleromyxedém bez monoklonální gamapatie"},"uk":{"name":"Склеромікседема без моноклональної гамопатії","definition":"Scleromyxedema without monoclonal gammopathy is a form of atypical lichen myxedematosus (see this term), characterized by a generalized sclerodermoid infiltration of skin studded with multiple, firm papules of 1-3 mm in diameter involving face (leonine appearance), trunk, and limbs, without monoclonal gammopathy. The involvement of the face can be missing and pruritus may be prominent."},"zh":{"name":"硬化性粘液水肿不伴单克隆丙种球蛋白病"},"tr":{"name":"Monoklonal gammopati olmayan skleromiksödem"}},"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":"0019585","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681463","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":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":15.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}