{"orpha":280065,"name":"Calciphylaxis cutis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, life-threatening, non-inflammatory vasculopathy characterized clinically by progressive and painful skin lesions associated with calcification of cutaneous arterial microvessels. Calciphylaxis predominantly affects patients with end-stage kidney disease (ESKD) on dialysis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-280065/","orphanet_url":"https://www.orpha.net/en/disease/detail/280065","names":{"fr":{"name":"Calciphylaxie cutanée","definition":"Vasculopathie non inflammatoire rare, potentiellement létale, caractérisée sur le plan clinique par des lésions cutanées progressives et douloureuses associées à une calcification des microvaisseaux artériels cutanés. La maladie touche principalement les patients atteints d'insuffisance rénale terminale (IRT) sous dialyse."},"de":{"name":"Kalziphylaxie, kutane","definition":"Eine seltene, lebensbedrohliche, nicht-entzündliche Vaskulopathie, die klinisch durch fortschreitende und schmerzhafte Hautläsionen gekennzeichnet ist, die mit einer Verkalkung der kutanen arteriellen Mikrogefäße einhergehen. Die Calciphylaxie betrifft vor allem Patienten mit Nierenerkrankungen im Endstadium (ESKD), die an der Dialyse behandelt werden."},"es":{"name":"Calcifilaxis cutánea","definition":"Es una vasculopatía no inflamatoria poco frecuente y potencialmente mortal que se caracteriza clínicamente por lesiones cutáneas progresivas y dolorosas asociadas a la calcificación de los microvasos arteriales cutáneos. La calcifilaxis afecta predominantemente a pacientes con enfermedad renal terminal (ERT) en diálisis."},"it":{"name":"Calcifilassi cutanea","definition":"Si tratta di una vasculopatia non infiammatoria rara, potenzialmente letale, caratterizzata dal punto di vista clinico da lesioni cutanee progressive e dolorose, associate alla calcificazione dei microvasi arteriosi della cute. La calcifilassi colpisce prevalentemente i pazienti con malattia renale terminale (ESKD) sottoposti a dialisi."},"nl":{"name":"Calciphylaxis cutis","synonyms":["Cutane calcifylaxie"],"definition":"Een zeldzame, levensbedreigende niet-inflammatoire vasculopathie, klinisch gekenmerkt door progressieve en pijnlijke huidlaesies geassocieerd met calcificatie van cutane arteriële microvaten. Calciphylaxis treft vooral patiënten met terminaal nierfalen (ESRD) die dialyse ondergaan."},"pt":{"name":"Calcifilaxia cutis","definition":"Vasculopatia não inflamatória rara, com risco de ser fatal, caracterizada clinicamente por lesões cutâneas progressivas e dolorosas associadas à calcificação dos microvasos arteriais cutâneos. A calcifilaxia afeta predominantemente os doentes com doença renal em estágio terminal (ESKD) e em diálise."},"pl":{"name":"Kalcyfilaksja skóry"},"cs":{"name":"Calciphylaxis cutis"},"uk":{"name":"Кальцифілаксія шкіри","definition":"A rare, life-threatening, non-inflammatory vasculopathy characterized clinically by progressive and painful skin lesions associated with calcification of cutaneous arterial microvessels. Calciphylaxis predominantly affects patients with end-stage kidney disease (ESKD) on dialysis."},"zh":{"name":"皮肤钙化防御","definition":"皮肤钙化症是一种危及生命的综合征，其特征是进行性疼痛性皮肤溃疡，伴有中小型皮肤动脉血管钙化。主要影响透析或肾移植患者。"},"tr":{"name":"Kalsifilaksi kutis"}},"xrefs":[{"source":"ICD-10","code":"E83.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB90.42","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017216","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4274083","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":280062,"name":"Calciphylaxis","classifications":[196,198]},{"orpha":79379,"name":"Skin vascular disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}