{"orpha":48377,"name":"Subcorneal pustular dermatosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pustulosis subcornealis","Sneddon-Wilkinson disease","Subcorneal pustular dermatitis"],"definition":"A rare, acquired, chronic neutrophilic dermatosis characterized by sterile superficial pustules, typically involving the flexural sites of the trunk and proximal extremities.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-48377/","orphanet_url":"https://www.orpha.net/en/disease/detail/48377","names":{"fr":{"name":"Dermatose pustuleuse sous-cornée","synonyms":["Maladie de Sneddon-Wilkinson","Pustulosis subcornealis"],"definition":"Dermatose neutrophile chronique rare et acquise, caractérisée par des pustules superficielles stériles, touchant typiquement les zones des plis cutanés du tronc et les parties proximales des extrémités."},"de":{"name":"Dermatose, pustulöse subkorneale","synonyms":["Sneddon-Wilkinson-Syndrom","Subkorneale Pustulosis"],"definition":"Eine seltene, erworbene, chronische neutrophile Dermatose, die durch sterile oberflächliche Pusteln gekennzeichnet ist und typischerweise die Beugeseiten des Rumpfes und der proximalen Extremitäten betrifft."},"es":{"name":"Dermatosis pustulosa subcórnea","synonyms":["Dermatitis pustulosa subcórnea","Enfermedad de Sneddon-Wilkinson","Pustulosis subcórnea"],"definition":"Es una dermatosis neutrofílica crónica, adquirida y poco frecuente, caracterizada por pústulas superficiales estériles que afectan típicamente las superficies de flexión del tronco y de las extremidades proximales."},"it":{"name":"Dermatosi pustolosa subcorneale","synonyms":["Dermatite pustolosa subcorneale","Malattia di Sneddon-Wilkinson","Pustulosis subcornealis"]},"nl":{"name":"Subcorneale pustuleuze dermatose","synonyms":["Pustulosis subcornealis","Subcorneale pustuleuze dermatitis","Ziekte van Sneddon-Wilkinson"],"definition":"Een zeldzame, verworven, chronische neutrofiele dermatose, gekenmerkt door steriele superficiële pustels, doorgaans met betrokkenheid van buigzijden van de romp en proximale extremiteiten."},"pt":{"name":"Dermatose pustular sub-corneana","synonyms":["Doença de Sneddon-Wilkinson"],"definition":"Dermatose neutrofílica crónica, rara e adquirida, caracterizada por pústulas superficiais estéreis, geralmente envolvendo os locais flexurais do tronco e extremidades proximais."},"pl":{"name":"Podrogowa dermatoza krostkowa","synonyms":["Choroba Sneddona i Wilkinsona","Podrogowe krostkowe zapalenie skóry"]},"cs":{"name":"Subkorneální pustulární dermatóza","synonyms":["Pustulosis subcornealis","Sneddonova-Wilkinsonova nemoc","Subkorneální pustulární dermatitida","Dermatitis pustulosa subcornealis","Sneddon-Wilkinsonova nemoc"]},"uk":{"name":"Субкорнеальний пустульозний дерматоз","synonyms":["Пустульоз субкорнеальний","Хвороба Снеддона-Вілкінсона","Підшкірний пустульозний дерматит"],"definition":"Subcorneal pustular dermatosis is a rare, benign, chronic disease characterized by sterile pustular eruption, typically involving the flexural sites of the trunk and proximal extremities."},"zh":{"name":"角质层下脓疱皮肤病","synonyms":["角膜下脓疱性皮炎"]},"tr":{"name":"Subkorneal püstüler dermatoz","synonyms":["Püstülozis subkornealis","Sneddon-Wilkinson hastalığı","Subkorneal püstüler dermatit"]}},"xrefs":[{"source":"ICD-10","code":"L13.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EB2Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1952122675"},{"source":"MONDO","code":"0006614","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0600336","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13606","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10042342","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":90077,"name":"Other acquired skin disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":200.0,"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":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000953","label":"Hyperpigmentation of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000836","label":"Hyperthyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000821","label":"Hypothyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010702","label":"Increased circulating immunoglobulin concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006775","label":"Multiple myeloma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001370","label":"Rheumatoid arthritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002725","label":"Systemic lupus erythematosus","frequency":"Occasional (29-5%)","criteria":null}]}}