{"orpha":529864,"name":"Secondary erythromelalgia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Secondary erythermalgia"],"definition":"A rare neurologic disease characterized by intermittent pain, erythema, swelling, and heat of the extremities which is aggravated by warming and improved by cooling. Attacks can last between minutes and days and tend to be precipitated by heat, exercise, or physical dependence. The disease may be linked to a variety of underlying conditions including hematological, metabolic, connective tissue or neurological disorders, neoplasia, infections, and certain drug side effects, among others.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-529864/","orphanet_url":"https://www.orpha.net/en/disease/detail/529864","names":{"fr":{"name":"Erythromélalgie secondaire","synonyms":["Erythermalgie secondaire"],"definition":"Maladie neurologique rare caractérisée par une douleur intermittente, un érythème, un oedème et une sensation de chaleur dans les extrémités aggravée par le réchauffement et améliorée par le refroidissement. Les crises peuvent durer entre quelques minutes et quelques jours et semblent être provoquées par la chaleur, l'exercice ou la dépendance physique. La maladie peut être liée à diverses maladies sous-jacentes, notamment des troubles hématologiques, métaboliques, du tissu conjonctif ou neurologiques, des néoplasies, des infections et certains effets secondaires de médicaments, entre autres."},"de":{"name":"Sekundäre Erythromelalgie","definition":"Eine seltene neurologische Erkrankung, die durch intermittierende Schmerzen, Rötungen, Schwellungen und Hitze der Extremitäten gekennzeichnet ist, die sich durch Erwärmung verschlimmern und durch Abkühlung bessern. Die Anfälle können zwischen Minuten und Tagen dauern und werden in der Regel durch Hitze, Bewegung oder körperliche Abhängigkeit ausgelöst. Die Krankheit kann mit einer Vielzahl von Grunderkrankungen in Verbindung gebracht werden, unter anderem mit hämatologischen, metabolischen, bindegewebigen oder neurologischen Störungen, Neoplasien, Infektionen und bestimmten Arzneimittelnebenwirkungen."},"es":{"name":"Eritromelalgia secundaria","synonyms":["Eritermalgia secundaria"],"definition":"Es una enfermedad neurológica poco frecuente caracterizada por dolor intermitente, eritema, inflamación y calor en las extremidades que se agrava con el calor y mejora con el enfriamiento. Las crisis pueden durar entre minutos y días y tienden a estar desencadenadas por el calor, el ejercicio o la dependencia física. La enfermedad puede estar asociada a una variedad de trastornos subyacentes que incluyen trastornos hematológicos, metabólicos, del tejido conectivo o neurológicos, neoplasia, infecciones y efectos secundarios de medicamentos, entre otros."},"it":{"name":"Eritromelalgia secondaria"},"nl":{"name":"Secundaire erytromelalgie","synonyms":["Secundaire erythermalgie"],"definition":"Een zeldzame neurologische aandoening, gekenmerkt door intermitterende pijn, erytheem, zwelling, en warme extremiteiten met verergering bij opwarming en verbetering bij afkoeling. Aanvallen kunnen minuten tot dagen duren, en verergeren door hitte, inspanning, of lichamelijke afhankelijkheid. De ziekte kan gekoppeld zijn aan verschillende onderliggende aandoeningen, waaronder hematologische, metabole of neurologische aandoening, bindweefselziekte, neoplasie, infectie, en bijwerkingen van bepaalde medicatie."},"pt":{"name":"Eritromelalgia secundária","definition":"Doença neurológica rara caracterizada por dor intermitente, eritema, edema e calor nas extremidades, agravado pelo aumento da temperatura e que melhora com o arrefecimento. As crises podem durar minutos ou dias e tendem a ser precipitadas por calor, exercício ou dependência física. A doença pode estar ligada a uma variedade de situações clínicas subjacentes, incluindo condições hematológicas, metabólicas, do tecido conjuntivo ou neurológicas, neoplasia, infeções e determinados efeitos secundários de medicamentos, entre outros."},"pl":{"name":"Wtórna erytromelalgia","synonyms":["Wtórny bolesny rumień"],"definition":"Rzadka choroba neurologiczna charakteryzująca się okresowym bólem, rumieniem, obrzękiem i uciepleniem kończyn, które nasilają się po ogrzaniu i zmniejszają po ochłodzeniu. Ataki mogą trwać od kilku minut do kilku dni i zwykle są wywoływane przez upał, wysiłek fizyczny lub uzależnienie. Choroba może być powiązana z różnymi schorzeniami podstawowymi, w tym między innymi chorobami hematologicznymi, metabolicznymi, tkanki łącznej lub neurologicznymi, nowotworami, infekcjami i działaniami niepożądanymi niektórych leków."},"cs":{"name":"Sekundární erytromelalgie","synonyms":["Secondary erythermalgia"]},"uk":{"name":"Вторинна еритромелалгія","synonyms":["Вторинна еритема"],"definition":"A rare neurologic disease characterized by intermittent pain, erythema, swelling, and heat of the extremities which is aggravated by warming and improved by cooling. Attacks can last between minutes and days and tend to be precipitated by heat, exercise, or physical dependence. The disease may be linked to a variety of underlying conditions including hematological, metabolic, connective tissue or neurological disorders, neoplasia, infections, and certain drug side effects, among others."},"zh":{"name":"继发性红斑性肢痛病","synonyms":["Secondary erythermalgia"]},"tr":{"name":"Sekonder eritromelalji","synonyms":["Sekonder eritmalji"]}},"xrefs":[{"source":"ICD-10","code":"I73.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EG00","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1009405597"},{"source":"MONDO","code":"0035149","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0543820","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":496924,"name":"Non-inflammatory vasculopathy","classifications":[198]},{"orpha":79379,"name":"Skin vascular disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.2,"area":"United States","status":"Validated","source":"18713229[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2026-05-19","source":"26419464[PMID]_10724194[PMID]_23762561[PMID]_29641704[PMID]_30609105[PMID]","terms":[{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032147","label":"Erythromelalgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000970","label":"Anhidrosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033665","label":"Diminished health-related quality of life","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001872","label":"Abnormality of thrombocytes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001063","label":"Acrocyanosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012533","label":"Allodynia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002936","label":"Distal sensory impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012534","label":"Dysesthesia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002045","label":"Hypothermia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001909","label":"Leukemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005547","label":"Myeloproliferative disorder","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002664","label":"Neoplasm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010741","label":"Pedal edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001901","label":"Polycythemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002633","label":"Vasculitis","frequency":"Occasional (29-5%)","criteria":null}]}}