{"orpha":535,"name":"Rare cutaneous lupus erythematosus","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"Rare cutaneous lupus erythematosus (CLE) is an autoimmune disease that denotes a heterogeneous spectrum of clinical manifestations affecting the skin and can be divided into 4 categories: acute CLE (ACLE); subacute CLE (SCLE); chronic CLE (CCLE; the most diverse form); and intermittent CLE (ICLE). CLE can either occur alone or associated with systemic lupus erythematosus (SLE).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-535/","orphanet_url":"https://www.orpha.net/en/disease/detail/535","names":{"fr":{"name":"Lupus érythémateux cutané rare","definition":"Le lupus érythémateux cutané rare (LED) est une maladie auto-immune qui dénote un spectre hétérogène de manifestations cliniques affectant la peau et peut être divisé en 4 catégories : le LED aigu (ACLE) ; le LED subaigu (SCLE) ; le LED chronique (CCLE ; la forme la plus diverse) ; et le LED intermittent (ICLE). Le LED peut se produire seul ou associé à un lupus érythémateux systémique (LED)."},"de":{"name":"Lupus erythematodes, kutaner seltener","definition":"Der kutane Lupus erythematodes (CLE) ist eine seltene Autoimmunerkrankung, die ein heterogenes Spektrum klinischer Manifestationen an der Haut aufweist und in vier Kategorien unterteilt werden kann: akuter CLE (ACLE), subakuter CLE (SCLE), chronischer CLE (CCLE; die vielfältigste Form) und intermittierender CLE (ICLE). CLE kann entweder allein oder in Verbindung mit systemischem Lupus erythematosus (SLE) auftreten."},"es":{"name":"Lupus eritematoso cutáneo raro","definition":"Es una enfermedad autoinmune que presenta un espectro heterogéneo de manifestaciones clínicas de afectación cutánea. Se puede clasificar en 4 categorías: LEC Agudo (LECA); LEC Subagudo (LECS); LEC Crónico (LECC; la forma más diversa); y LEC Intermitente (LECI). LEC puede manifestarse de forma aislada o asociado a lupus eritematoso sistémico (LES)."},"it":{"name":"Lupus eritematoso cutaneo"},"nl":{"name":"Zeldzame cutane lupus erythematosus","definition":"Zeldzame cutane lupus erythematosus (CLE) is een auto-immuunziekte die duidt op een heterogeen spectrum van klinische manifestaties aan de huid en die kan onderverdeeld worden in 4 categorieën: acute CLE (ACLE); subacute CLE (SCLE); chronische CLE (CCLE; de meest diverse vorm); en intermitterende CLE (ICLE). CLE kan alleen voorkomen of geassocieerd zijn met systemische lupus erythematosus (SLE)."},"pt":{"name":"Lúpus eritematoso cutâneo raro","definition":"O lúpus eritematoso cutâneo (LEC) raro é uma doença autoimune que apresenta um espectro heterogéneo de manifestações clínicas que afetam a pele e pode ser dividida em 4 categorias: LEC agudo (LECA); LEC subagudo (LECS); LEC crónico (LECC; a forma mais diversa); e LEC intermitente (LECI). O LEC pode ocorrer isoladamente ou associado a lúpus eritematoso sistémico (LES)."},"pl":{"name":"Rzadka postać tocznia rumieniowatego skórnego"},"cs":{"name":"Kožní lupus erythematodes"},"uk":{"name":"Рідкісний шкірний червоний вовчак","definition":"Rare cutaneous lupus erythematosus (CLE) is an autoimmune disease that denotes a heterogeneous spectrum of clinical manifestations affecting the skin and can be divided into 4 categories: acute CLE (ACLE); subacute CLE (SCLE); chronic CLE (CCLE; the most diverse form); and intermittent CLE (ICLE). CLE can either occur alone or associated with systemic lupus erythematosus (SLE)."},"zh":{"name":"罕见皮肤红斑狼疮"},"tr":{"name":"Nadir kutanöz lupus eritematozus"}},"xrefs":[{"source":"MONDO","code":"18887","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680424","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6225","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10056509","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":46489,"name":"OBSOLETE: Bullous systemic lupus erythematosus","status":"inactive"},{"type":"Referred to","orpha":90287,"name":"OBSOLETE: Maculopapular lupus rash","status":"inactive"},{"type":"Referred to","orpha":163528,"name":"OBSOLETE: Acute cutaneous lupus erythematosus","status":"inactive"}],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":315350,"name":"Autoimmune disease with skin involvement","classifications":[187]}],"children":[{"orpha":163531,"name":"Chronic cutaneous lupus erythematosus","type":"Clinical group"},{"orpha":163525,"name":"Subacute cutaneous lupus erythematosus","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":50.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2007[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.3,"area":"United States","status":"Validated","source":"19289752[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"6-9 / 10 000","value":73.0,"area":"United States","status":"Validated","source":"19289752[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.0,"area":"Sweden","status":"Validated","source":"21574972[PMID]"}],"phenotypes":null}