{"orpha":83452,"name":"Complex regional pain syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Sudeck's atrophy","Causalgia","Reflex sympathetic dystrophy","Algodystrophy","Algoneurodystrophy"],"definition":"A rare neurologic disease characterized by persistent pain that is disproportionate to the inciting event. The pain is regional, usually affecting a limb with a distal predominance, and may occur spontaneously or be stimulus-induced. It is associated with sensory disturbances, vasomotor abnormalities (including changes in skin color and temperature), sudomotor abnormalities, edema, and motor or trophic changes. Symptoms often develop following trauma, fractures, immobilization, surgery, or nerve injury, and their severity and clinical presentation can vary considerably among patients.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83452/","orphanet_url":"https://www.orpha.net/en/disease/detail/83452","names":{"fr":{"name":"Syndrome douloureux régional complexe","synonyms":["Dystrophie sympathique réflexe","Algodystrophie","Algoneurodystrophie","Atrophie de Sudeck","Causalgie"],"definition":"Maladie neurologique rare et évolutive qui regroupe un ensemble de maladies caractérisées par une douleur disproportionnée, spontanée ou provoquée par un stimulus, accompagnée de nombreux troubles, plus ou moins variés, affectant le système nerveux autonome et le système moteur, avec pour symptômes l'oedème, l'allodynie, ainsi que des troubles de l'irrigation sanguine et des troubles trophiques. Le SDRC touche le plus souvent l'un des bras, des jambes, des mains ou des pieds et survient généralement à la suite d'une blessure ou d'un traumatisme au niveau du membre concerné."},"de":{"name":"Komplexes regionales Schmerzsyndrom","synonyms":["Kausalgie","Sudeck-Atrophie","CRPS"],"definition":"Das komplexe regionale Schmerzsyndrom (CRPS) ist eine seltene neurologische Erkrankung mit schmerzhafter Progression, die einer Gruppe von Erkrankungen umfasst, die durch einen unverhältnismäßig starken spontanen oder durch einen Reiz verursachten Schmerz gekennzeichnet ist. CRPS ist begleitet von einer Vielzahl variabel gemischter autonomer und motorischer Beeinträchtigungen einschließlich Symptomen wie Schwellung, Allodynie, Durchblutungsstörungen und trophischen Störungen. Die CRPS betrifft meist einen der Arme, Beine, Hände oder Füße und tritt häufig nach einer Verletzung oder einem Trauma dieser Extremität auf."},"es":{"name":"Síndrome de dolor regional complejo","synonyms":["Algoneurodistrofia","Atrofia de Sudeck","Causalgia","Distrofia simpática refleja","Algodistrofia"],"definition":"El síndrome de dolor regional complejo (SDRC) es una enfermedad neurológica dolorosa progresiva poco común que corresponde a un grupo de trastornos caracterizados por dolor desproporcionado, espontáneo o inducido por estímulos, acompañado de una miríada variable de trastornos autonómicos y motores que incluyen síntomas como hinchazón, alodinia, irrigación cutánea y alteraciones tróficas. El SDRC afecta con mayor frecuencia a los brazos, piernas, manos o pies de forma unilateral y suele ocurrir después de una lesión o trauma en esa extremidad."},"it":{"name":"Sindrome da dolore regionale complesso","synonyms":["Causalgia","Distrofia simpatica riflessa","Algodistrofia","Atrofia di Sudeck","Algoneurodistrofia"]},"nl":{"name":"Complex regionaal pijnsyndroom","synonyms":["Atrofie van Sudeck","Sudeck-atrofie","Causalgie","Sympathische reflexdystrofie","Algodystrofie","Algoneurodystrofie","CRPS"],"definition":"Complex regionaal pijnsyndroom (CRPS) is een zeldzame, neurologische, pijnlijke en progressieve conditie die overeenkomt met een groep aandoeningen die worden gekarakteriseerd door een onevenredige spontane of door een prikkel uitgelokte pijn, samen met een groot aantal variabele combinaties van autonome en motorische aandoeningen waaronder symptomen zoals zwellingen, allodynie, verstoorde aanvoer van bloed naar de huid, en trofische stoornissen. CRPS treft meestal één van de armen, benen, handen of voeten, en komt doorgaans voor na een blessure of verwonding."},"pt":{"name":"Síndrome dor regional complexa"},"pl":{"name":"Zespół złożonego bólu miejscowego","synonyms":["Sudeck's atrophy","Causalgia","Reflex sympathetic dystrophy","Algodystrophy","Algoneurodystrophy"]},"cs":{"name":"Komplexní regionální bolestivý syndrom","synonyms":["KRBS","Sudeckův syndrom"]},"uk":{"name":"Комплексний регіональний больовий синдром","definition":"Complex regional pain syndrome (CRPS) is a rare neurologic disease painful progressive condition that corresponds to a group of disorders characterized by a disproportionate spontaneous or stimulus-induced pain, accompanied by a variably mixed myriad of autonomic and motor disorders including symptoms such as swelling, allodynia, skin blood supply and trophic disturbances. CRPS most often affects one of the arms, legs, hands, or feet and usually occurs after an injury or trauma to that limb."},"zh":{"name":"复杂性局部疼痛综合征"},"tr":{"name":"Kompleks bölgesel ağrı sendromu"}},"xrefs":[{"source":"OMIM","code":"604335","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"G90.5","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"G90.6","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"MG30.04","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1834504950"},{"source":"MONDO","code":"0019369","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D020918","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0458219","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4647","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10064332","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":99994,"name":"OBSOLETE: Complex regional pain syndrome type 2","status":"inactive"},{"type":"Referred to","orpha":99995,"name":"OBSOLETE: Complex regional pain syndrome type 1","status":"inactive"}],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98006,"name":"Rare neurologic disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":26.2,"area":"Netherlands","status":"Validated","source":"17084977[PMID]_24161450[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-09-17","source":"10522900[PMID]","terms":[{"hpo":"HP:0009763","label":"Limb pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0040170","label":"Abnormality of hair growth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012533","label":"Allodynia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000958","label":"Dry skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012534","label":"Dysesthesia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010742","label":"Edema of the upper limbs","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004305","label":"Involuntary movements","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010741","label":"Pedal edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008383","label":"Slow-growing nails","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003474","label":"Somatic sensory dysfunction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010834","label":"Trophic changes related to pain","frequency":"Frequent (79-30%)","criteria":null}]}}