{"orpha":140933,"name":"Linear atrophoderma of Moulin","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Linear atrophoderma of Moulin (LAM) is characterized by mildly atrophic and hyperpigmented band-like lesions that follow the lines of Blaschko on the trunk or limbs. Since its initial description in 1992, less than 30 cases have been reported in the literature. Onset occurs during childhood or adolescence and the disease is non-progressive. There is no prior inflammation or subsequent scleroderma. The aetiology is unknown but as LAM follows the lines of Blaschko it has been suggested that the disease is caused by mosaicism of a predisposing gene.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-140933/","orphanet_url":"https://www.orpha.net/en/disease/detail/140933","names":{"fr":{"name":"Atrophodermie linéaire de Moulin","definition":"L'atrophodermie linéaire de Moulin (ALM) est caractérisée par des lésions légèrement atrophiques et hyperpigmentées disposées en bandes selon les lignes de Blaschko du tronc et des membres. Depuis sa première description en 1992, moins de 30 cas ont été rapportés dans la littérature. La maladie apparaît durant l'enfance ou l'adolescence. La maladie est non-progressive. Il n'y a pas d'inflammation précédant l'ALM, ni de sclérodermie ultérieure. L'étiologie reste inconnue mais, étant donné que l'ALM suit les lignes de Blaschko, elle pourrait être due à un mosaïcisme d'un gène de susceptibilité."},"de":{"name":"Atrophodermie, lineare, Typ Moulin","synonyms":["Atrophodermia linearis Moulin"],"definition":"Die Lineare Atrophodermie Typ Moulin (LAM) ist gekennzeichnet durch leicht atrophische, hyperpigmentierte bandförmige Veränderungen der Haut, die am Stamm und an den Gliedmaßen den Blaschko-Linien folgen."},"es":{"name":"Atrofodermia lineal de Moulin","definition":"El atrofoderma lineal de Moulin (ALM) se caracteriza por lesiones ligeramente atróficas e hiperpigmentadas dispuestas en bandas según las líneas de Blaschko del tronco y de las extremidades. Desde su primera descripción en 1992, se han descrito menos de 30 casos en la literatura. Aparece durante la infancia o la adolescencia y es una enfermedad no progresiva. No existe una inflamación que la preceda, ni una esclerodermia subsecuente. Se desconoce la etiología, pero como la ALM sigue las líneas de Blaschko, se ha sugerido que la enfermedad podría estar causada por un mosaicismo de un gen de susceptibilidad."},"it":{"name":"Atrofoderma lineare di Moulin"},"nl":{"name":"Lineaire atrofodermie van Moulin","definition":"Een aandoening, gekenmerkt door mild atrofische en hypergepigmenteerde band-achtige laesies volgens de lijnen van Blaschko op romp of ledematen. Sinds de initiële beschrijving in 1992 werden minder dan 30 gevallen van lineaire atrofodermie van Moulin (LAM) gerapporteerd in de literatuur. De ziekte vangt aan in de kindertijd of adolescentie en is niet-progressief. Er is geen voorafgaande inflammatie of navolgende sclerodermie. De etiologie is niet gekend, maar aangezien LAM de lijnen van Blaschko volgt, werd gesuggereerd dat de ziekte wordt veroorzaakt door mozaïcisme van een predispositiegen."},"pt":{"name":"Atrofodermia linear de Moulin","definition":"A atrofodermia linear de Moulin (LAM) é caracterizada por lesões ligeiramente atróficas e hiperpigmentadas dispostas em bandas, que seguem as linhas de Blaschko do tronco e dos membros."},"pl":{"name":"Linijna atrofodermia Moulina"},"cs":{"name":"Lineární Moulinova atrophoderma"},"uk":{"name":"Лінійна атрофодермія Мулена","definition":"Linear atrophoderma of Moulin (LAM) is characterized by mildly atrophic and hyperpigmented band-like lesions that follow the lines of Blaschko on the trunk or limbs. Since its initial description in 1992, less than 30 cases have been reported in the literature. Onset occurs during childhood or adolescence and the disease is non-progressive. There is no prior inflammation or subsequent scleroderma. The aetiology is unknown but as LAM follows the lines of Blaschko it has been suggested that the disease is caused by mosaicism of a predisposing gene."},"zh":{"name":"Moulin 线状皮肤萎缩","definition":"Moulin 线状皮肤萎缩（LAM）的特征是轻度萎缩和色素沉着的带状病变，该病变在躯干或四肢上沿Blaschko线分布。自1992年首次描述以来，文献报道的病例不到30例。在儿童或青少年期起病，且该病无进行性表现。无既往炎症或继发硬皮病。病因不明，但由于LAM沿Blaschko线分布，有人认为该病是由一个易感基因的嵌合体引起。"},"tr":{"name":"Lineer Moulin atrofoderması"}},"xrefs":[{"source":"ICD-10","code":"L90.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB61.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1116101506"},{"source":"MONDO","code":"0015371","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1274753","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19932","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79381,"name":"Other dermis disorder","classifications":[187]},{"orpha":477808,"name":"Other genetic dermis disorder","classifications":[156]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":30.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-10-12","source":"27051814[PMID]_23989408[PMID]","terms":[{"hpo":"HP:0007546","label":"Linear hyperpigmentation","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Excluded (0%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Excluded (0%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0100324","label":"Scleroderma","frequency":"Excluded (0%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0030053","label":"Stiff skin","frequency":"Excluded (0%)","criteria":"Diagnostic criterion"}]}}