{"orpha":168606,"name":"Seborrhea-like dermatitis with psoriasiform elements","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, genetic, epidermal disorder characterized by a chronic, diffuse, fine, scaly erythematous rash on the face (predominantly the chin, nasolabial folds, eyebrows), around the earlobes and over the scalp, associated with hyperkeratosis over elbows, knees, palms, soles and metacarpophalangeal joints, in the absence of associated rheumatological or neurological disorders. Cold weather, emotional stress and strenuous physical activity may exacerbate symptoms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-168606/","orphanet_url":"https://www.orpha.net/en/disease/detail/168606","names":{"fr":{"name":"Dermatite séborrhéïque-like avec des éléments psoriasiques","definition":"Maladie rare de l'épiderme d'origine génétique caractérisée par une éruption érythémateuse squameuse fine, diffuse et chronique affectant le visage (principalement le menton, les sillons nasogéniens et les sourcils), les bords du lobe de l'oreille et le cuir chevelu, associée à une hyperkératose affectant les coudes, les genoux, les paumes des mains et la plante des pieds, ainsi que les articulations métacarpophalangiennes, en l'absence de maladies rhumatologiques ou neurologiques associées. Le froid, le stress émotionnel et une activité physique intense peuvent exacerber les symptômes."},"de":{"name":"Seborrhö-ähnliche Dermatitis mit psoriasiformen Ausschlägen"},"es":{"name":"Dermatitis tipo seborreica con elementos psoriasiformes","definition":"Es un trastorno epidérmico, genético y poco frecuente, caracterizado por una erupción eritematosa, crónica, difusa, fina y escamosa en el rostro (predominantemente en la barbilla, pliegues nasolabiales, cejas), alrededor de los lóbulos de las orejas y en el cuero cabelludo, asociado con una hiperqueratosis en los codos, las rodillas, las palmas de las manos, las plantas de los pies y las articulaciones metacarpofalángicas, en ausencia de trastornos reumatológicos o neurológicos asociados. El clima frío, el estrés emocional y la actividad física extenuante pueden exacerbar los síntomas."},"it":{"name":"Dermatite simil-seborroica con elementi psoriasiformi","definition":"La dermatite simil-seborroica con elementi psoriasiformi è una malattia genetica rara dell'epidermide, caratterizzata da eruzioni eritematose diffuse croniche, sottili e desquamative localizzate sul viso (soprattutto sul mento, sulle pliche nasolabiali e sulle sopracciglia), attorno ai lobi delle orecchie e sul cuoio capelluto, associate ad ipercheratosi sui gomiti, sulle ginocchia, sul palmo delle mani e sulla pianta dei piedi e sulle articolazioni metacarpo-falangee. Non si associa a malattie reumatiche o neurologiche. Le basse temperature, lo stress emotivo e l'eccessiva attività fisica possono esacerbare i sintomi."},"nl":{"name":"Seborroe-achtige dermatitis met psoriasiforme elementen","definition":"Seborroe-achtige dermatitis met psoriasiforme elementen is een zeldzame, genetische, epidermale stoornis die gekarakteriseerd wordt door een chronische, diffuse, fijne, schilferige erythemateuze uitslag op het gelaat (vooral de kin, nasolabiale plooien, wenkbrauwen), rond de oorlellen en op de hoofdhuid, geassocieerd met hyperkeratose aan ellebogen, knieën, handpalmen, voetzolen en metacarpofalangeale gewrichten, in afwezigheid van geassocieerde reumatologische of neurologische stoornissen. Koud weer, emotionele stress en inspannende fysieke activiteit kunnen de symptomen verergeren."},"pt":{"name":"Dermatite seborreica-like com elementos psoriasiformes"},"pl":{"name":"Zapalenie skóry podobne do łojotokowego zapalenia skóry z elementami łuszczycopodobnymi"},"cs":{"name":"Seborrhea-like dermatitida s psoriaziformními prvky"},"uk":{"name":"Себорейний дерматит з псоріазоподібними елементами","definition":"A rare, genetic, epidermal disorder characterized by a chronic, diffuse, fine, scaly erythematous rash on the face (predominantly the chin, nasolabial folds, eyebrows), around the earlobes and over the scalp, associated with hyperkeratosis over elbows, knees, palms, soles and metacarpophalangeal joints, in the absence of associated rheumatological or neurological disorders. Cold weather, emotional stress and strenuous physical activity may exacerbate symptoms."},"zh":{"name":"脂溢性皮炎样皮炎伴银屑病样成分","definition":"脂溢性皮炎样银屑病是一种罕见的遗传性表皮疾病，其主要临床表现为面部（主要是下巴、鼻唇沟、眉毛）、耳垂周围和头皮上弥漫性的细小鳞状红斑皮疹，病程长。在没有相关风湿性疾病或神经性疾病的情况下，这些皮疹与肘部、膝盖、手掌、脚底和掌指关节的角化过度有关。寒冷、精神紧张和剧烈的体力活动可能会使症状加重。"},"tr":{"name":"Psoriasiform elementler ile seyreden sebore benzeri dermatit"}},"xrefs":[{"source":"OMIM","code":"610227","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"L21.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EA81.0","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"ICD-11","code":"EA81.1","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"ICD-11","code":"EA81.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0012446","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C565217","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1853258","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":183426,"name":"Genetic epidermal disorder","classifications":[156]},{"orpha":79359,"name":"Other epidermal disorder","classifications":[187]}],"children":[],"genes":[{"symbol":"ZNF750","name":"zinc finger protein 750","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"16751772[PMID]","hgnc":"25843","ensembl":"ENSG00000141579","omim":"610226","loci":["17q25.3"]}],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":44.0,"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}