{"orpha":163927,"name":"Pustulosis palmaris et plantaris","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Palmoplantar pustulosis","LPP","Localized pustular psoriasis","PPP"],"definition":"A rare skin disease characterized by chronic eruption of sterile pustules on an erythematous and desquamative background. The lesions are usually painful and affect the palms and soles, sometimes also the lateral aspects of hands and feet. Nail lesions (such as nail pitting, onycholysis, subungual pustules, and nail dystrophy) are also observed. The condition takes a chronic and relapsing course. Typical associations are psoriatic arthritis, thyroid gland dysfunction, and smoking.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-163927/","orphanet_url":"https://www.orpha.net/en/disease/detail/163927","names":{"fr":{"name":"Pustulose palmoplantaire","synonyms":["PPL","PPP","Psoriasis pustuleux localisé"],"definition":"Maladie rare de la peau caractérisée par une éruption chronique de pustules stériles sur un fond érythémateux et desquamatif. Les lésions sont généralement douloureuses et siègent sur les paumes et la plante des pieds, parfois sur les faces latérales des mains et des pieds. On observe également des lésions des ongles (telles que des piqûres d'ongles, une onycholyse, des pustules sous-unguéales et une dystrophie unguéale). La maladie évolue de manière chronique et récurrente. Les associations typiques sont le rhumatisme psoriasique, le dysfonctionnement de la glande thyroïdienne et le tabagisme."},"de":{"name":"Pustulosis Palmaris et Plantaris","definition":"Eine seltene Hauterkrankung, die durch einen chronischen Ausbruch von sterilen Pusteln auf einem erythematösen und schuppenden Hintergrund gekennzeichnet ist. Die Läsionen sind in der Regel schmerzhaft und betreffen die Handflächen und Fußsohlen, manchmal auch die Seitenflächen der Hände und Füße. Auch Nagelläsionen (z. B. Nagelflecken, Onycholyse, subunguale Pusteln und Nageldystrophie) werden beobachtet. Die Erkrankung verläuft chronisch und schubweise. Typische Assoziationen sind Psoriasis-Arthritis, Schilddrüsenfehlfunktion und Rauchen."},"es":{"name":"Pustulosis palmar y plantar","synonyms":["PPP","Psoriasis pustulosa localizada","Pustulosis palmoplantar"],"definition":"Es una enfermedad cutánea poco frecuente caracterizada por unas lesiones crónicas consistentes en pústulas estériles sobre un fondo eritematoso y descamativo. Las lesiones suelen ser dolorosas y afectan a las palmas y a las plantas, y, ocasionalmente, a las caras laterales de las manos y los pies. También se observan lesiones ungueales (punteado, onicólisis, pústulas subungueales y distrofia ungueal). La entidad sigue un curso crónico y recurrente. Las asociaciones típicas son con la artritis psoriásica, una disfunción de la glándula tiroides y el tabaquismo."},"it":{"name":"Pustolosi palmo-plantare","synonyms":["LPP","PPP","Psoriasi pustolosa localizzata","Pustolosi palmoplantare"],"definition":"Si tratta di una malattia rara della cute, caratterizzata dall'eruzione cronica di pustole sterili su porzioni di cute eritematosa e desquamata. Di solito le lesioni sono dolorose e si localizzano sul palmo delle mani e sulla pianta dei piedi, e talvolta anche sulla porzione laterale delle mani e dei piedi. I pazienti presentano anche lesioni delle unghie (fossette, onicolisi, pustole subungueali e distrofia). Il decorso è cronico e recidivante. In genere si associa all'artrite psoriasica, alla disfunzione della tiroide e al fumo."},"nl":{"name":"Pustulosis palmaris et plantaris","synonyms":["Gelokaliseerde pustuleuze psoriasis","LPP","PPP","Palmoplantaire pustulose"],"definition":"Een zeldzame aandoening van huid, gekarakteriseerd door chronische eruptie van steriele pustels op een erythemateuze en schilferende achtergrond. De laesies zijn meestal pijnlijk en tasten handpalmen en voetzolen aan, en soms ook de laterale zijde van handen en voeten. Laesies van nagels (zoals putjesnagels, onycholyse, subunguale pustels, en nageldystrofie) worden ook waargenomen. De aandoening vertoont een chronisch en recidiverend verloop. Typische associaties zijn psoriatische artritis, disfunctie van schildklier, en roken."},"pt":{"name":"Pustulosis palmaris et plantaris"},"pl":{"name":"Łuszczyca krostkowa dłoni i podeszw","synonyms":["Krostkowica dłoni i podeszw","LPP","Ograniczona łuszczyca krostkowa","PPP"]},"cs":{"name":"Pustulosis palmaris et plantaris","synonyms":["LPP","Lokalizovaná pustulózní psoriáza","PPP","Palmoplantární pustulóza"]},"uk":{"name":"Пустульоз долонь і підошов","synonyms":["Локалізований пустульозний псоріаз","LPP","Пальмоплантарний пустульоз","РРР"],"definition":"A rare skin disease characterized by chronic eruption of sterile pustules on an erythematous and desquamative background. The lesions are usually painful and affect the palms and soles, sometimes also the lateral aspects of hands and feet. Nail lesions (such as nail pitting, onycholysis, subungual pustules, and nail dystrophy) are also observed. The condition takes a chronic and relapsing course. Typical associations are psoriatic arthritis, thyroid gland dysfunction, and smoking."},"zh":{"name":"掌跖脓疱病","synonyms":["局限性脓疱型银屑病"]},"tr":{"name":"Püstüloz palmaris et plantaris","synonyms":["LPP","PPP","Palmoplantar püstüloz","Lokalize püstüler sedef hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"L40.3","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EA90.42","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"877172115"},{"source":"MONDO","code":"0015597","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0030246","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12820","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10050185","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79359,"name":"Other epidermal disorder","classifications":[187]},{"orpha":79360,"name":"Other genetic epidermal disease","classifications":[156]}],"children":[],"genes":[{"symbol":"AP1S3","name":"adaptor related protein complex 1 subunit sigma 3","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"24791904[PMID]","hgnc":"18971","ensembl":"ENSG00000152056","omim":"615781","loci":["2q36.1"]},{"symbol":"IL36RN","name":"interleukin 36 receptor antagonist","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"23303454[PMID]","hgnc":"15561","ensembl":"ENSG00000136695","omim":"605507","loci":["2q14.1"]}],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Autosomal recessive"],"onset":[],"prevalence":[],"phenotypes":null}