{"orpha":222,"name":"Erosive pustular dermatosis of the scalp","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Erosive pustular dermatosis of the scalp is a rare chronic inflammation of the scalp usually occurring in elderly women (>70 years old) and characterized by the development of painful pustules, shallow erosions, and crusting on atrophic skin that eventually result in cicatricial alopecia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-222/","orphanet_url":"https://www.orpha.net/en/disease/detail/222","names":{"fr":{"name":"Dermatose pustuleuse érosive du cuir chevelu","definition":"La dermatose pustuleuse érosive du cuir chevelu est une inflammation chronique et rare du cuir chevelu survenant généralement chez les femmes âgées (> 70 ans). Elle se caractérise par le développement de pustules douloureuses, d'érosions peu profondes et de croûtes sur une peau atrophique, ce qui conduit finalement à une alopécie cicatricielle."},"de":{"name":"Erosiv-pustulöse Dermatose der Kopfhaut","definition":"Die Erosiv-pustulöse Dermatose der Kopfhaut ist eine seltene chronische Entzündung der Kopfhaut, die in der Regel bei älteren Frauen (>70 Jahre alt) auftritt. Charakteristisch ist die Entwicklung schmerzhafter Pusteln, flacher Erosionen und Verkrustungen auf der atrophischen Haut, die eventuell zu narbiger Alopezie führt."},"es":{"name":"Dermatosis pustulosa erosiva de cuero cabelludo","definition":"La dermatosis pustulosa erosiva de cuero cabelludo es una inflamación crónica poco frecuente del cuero cabelludo que normalmente ocurre en mujeres mayores (>70 años de edad) y que se caracteriza por el desarrollo de pústulas dolorosas, erosiones superficiales, y formación de costras en la piel atrófica que finalmente dan lugar a una alopecia cicatricial."},"it":{"name":"Dermatosi pustolosa erosiva del cuoio capelluto","definition":"La dermatosi pustolosa erosiva del cuoio capelluto è un'infiammazione cronica rara del cuoio capelluto, che di solito si manifesta nelle donne anziane (> 70 anni) ed è caratterizzata dallo sviluppo di pustole dolorose, erosioni superficiali e croste sulla cute atrofica, che alla fine esitano nell'alopecia cicatriziale."},"nl":{"name":"Erosieve pustuleuze dermatose van schedelhuid","definition":"Erosieve pustuleuze dermatose van schedelhuid is een zeldzame chronische inflammatie van scalp, die meestal voorkomt bij oudere vrouwen (ouder dan 70 jaar), en gekenmerkt wordt door ontwikkeling van pijnlijke pustels, oppervlakkige erosies, en korstvorming op atrofische huid die uiteindelijk resulteren in cicatriciële alopecie."},"pt":{"name":"Dermatose pustular erosiva do couro cabeludo"},"pl":{"name":"Nadżerkowe krostkowe zapalenie skóry głowy"},"cs":{"name":"Erozivní pustulární dermatóza skalpu"},"uk":{"name":"Ерозивно-пустульозний дерматоз волосистої частини голови","definition":"Erosive pustular dermatosis of the scalp is a rare chronic inflammation of the scalp usually occurring in elderly women (>70 years old) and characterized by the development of painful pustules, shallow erosions, and crusting on atrophic skin that eventually result in cicatricial alopecia."},"zh":{"name":"头皮糜烂性脓疱性皮病"},"tr":{"name":"Kafa derisinin erozif püstüler dermatozu"}},"xrefs":[{"source":"ICD-10","code":"L98.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EG30.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"982719772"},{"source":"MONDO","code":"0016379","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0406464","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2188","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":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001595","label":"Abnormality of the hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0004552","label":"Scarring alopecia of scalp","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Frequent (79-30%)","criteria":null}]}}