{"orpha":254367,"name":"Rare lichen planus","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["Rare LP"],"definition":"Lichen planus (LP) is a common inflammatory dermatosis characterized by the development of pruritic violaceous papules or plaques on mucocutaneous surfaces. Eruptions can involve the face, neck, limbs, back, genitalia, tongue, buccal mucosa, nails, and scalp. LP comprises rare variants affecting the skin and the mucosa. Rare cutaneous LP includes linear LP (referring to blaschkoid and zosteriform distributions of lichenoid lesions), actinic LP, annular LP, atrophic LP, annular atrophic LP, lichen planopilaris (comprising Graham Little-Piccardi-Lassueur syndrome and frontal fibrosing alopecia), lichen planus pigmentosus, and lichen planus pemphigoides. Rare mucosal LP includes vulvovaginal gingival syndrome and LP sialadenitis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-254367/","orphanet_url":"https://www.orpha.net/en/disease/detail/254367","names":{"fr":{"name":"Lichen plan rare","synonyms":["LP rare"],"definition":"Le lichen plan (LP) est une dermatose inflammatoire fréquente caractérisée par l'apparition de papules ou de plaques violines prurigineuses cutanéomuqueuses. L'éruption peut toucher la face, le cou, les membres le dos, les organes génitaux, la langue, la muqueuse buccale, les ongles et le cuir chevelu. Le LP comprend des variantes rares ne touchant que la peau ou les muqueuses. Le LP cutané comprend le LP linéaire (désignant des lésions de distribution blaschkoïde et zostériforme), le LP actinique, Le LP annulaire, le LP atrophique, Le LP annulaire atrophique, le LP pilaire (comprenant le syndrome de Graham-Little-Piccardi-Lassueur et l'alopécie frontale fibrosante), le LP pigmentaire et le LP pemphigoïde. Le LP muqueux comprend le le LP buccal et le syndrome vulvo-vaginal-gingival."},"de":{"name":"Lichen planus, seltener","synonyms":["LP, seltene Form"],"definition":"Der Lichen planus (LP) ist eine häufige entzündliche Dermatose und gekennzeichnet durch die Entwicklung juckender livider Papeln oder Plaques auf den muko-kutanen Oberflächen von Gesicht, Hals, Gliedmaßen, Rücken, Genitalien, Zunge, Mundschleimhaut, Nägeln und Kopfhaut. Zum LP gehören auch seltene Varianten der Haut und der Mukosa. Formen des seltenen kutanen LP sind der Lineare LP (benannt nach der Verteilung der lichenoiden Läsionen, die Blaschko-Linien oder Zoster ähnelt), aktinischer LP, annulärer LP, atrophischer LP, annulär-atrophischer LP, Lichen planopilaris (mit dem Graham Little-Piccardi-Lassueur-Syndrom und der Frontalen fibrosierenden Alopezie), Lichen planus pigmentosus und Lichen planus pemphigoides (s. diese Termini). Formen des seltenen mukösen LP sind das Vulvo-vaginal-gingival-Syndrom und die LP-Sialadenitis (s. diese Termini)."},"es":{"name":"Liquen plano raro","definition":"El liquen plano (LP) es una dermatosis inflamatoria común caracterizada por el desarrollo de pápulas pruriginosas violáceas o placas en las superficies mucocutáneas. Las erupciones pueden afectar a cara, cuello, extremidades, espalda, genitales, lengua, mucosa bucal, uñas y cuero cabelludo. El LP comprende variantes raras que afectan a la piel y la mucosa. Los LP cutáneos raros incluyen: LP lineal (referido a una distribución de las lesiones liquenoides zosteriforme y siguiendo las líneas de Blaschko), LP actínico, LP anular, LP atrófico, LP atrófico anular, liquen plano pilar (incluidos el síndrome de Graham Little-Piccardi-Lassueur y la alopecia frontal fibrosante), liquen plano pigmentoso, y liquen plano penfigoide (consulte estos términos). Entre los LP de la mucosa raros se incluyen el síndrome vulvovaginal-gingival y el LP con sialadenitis (consulte estos términos)."},"it":{"name":"Lichen planus raro","synonyms":["LP raro"],"definition":"Il lichen piano (LP) è una comune dermatosi infiammatoria, caratterizzata dallo sviluppo di papule o placche violacee pruriginose sulle superfici mucocutanee. Le eruzioni possono interessare la faccia, il collo, gli arti, la schiena, i genitali, la lingua, la mucosa orale, le unghie e il cuoio capelluto. Il LP comprende alcune varianti rare, che interessano la cute e le mucose. Il LP cutaneo raro si distingue in LP lineare (che si associa a una disposizione blaschkoide e zosteriforme delle lesioni lichenoidi), LP attinico, LP anulare, LP atrofico, LP atrofico anulare, lichen piano pilare (compresa la sindrome di Graham Little-Piccardi-Lassueur e l'alopecia fibrosante frontale), lichen piano pigmentato e lichen piano pemfigoide (si vedano questi termini). Il LP raro della mucosa comprende la sindrome gengivale vulvo-vaginale e il LP con scialoadenite (si vedano questi termini)."},"nl":{"name":"Zeldzame lichen planus","synonyms":["Zeldzame LP"],"definition":"Lichen planus (LP) is een vaak voorkomende inflammatoire dermatose gekenmerkt door de ontwikkeling van jeukende, violetkleurige papels of plaques op mucocutane oppervlakken. Er kunnen erupties optreden in het aangezicht, de hals, de ledematen, de rug, de genitaliën, de tong, het mondslijmvlies, de nagels en de hoofdhuid. LP omvat zeldzame varianten die de huid en het slijmvlies treffen. Zeldzame cutane LP omvat lineaire LP (verwijzend naar Blaschkoïde en zosteriforme verdelingen van lichenoïde laesies), actinische LP, annulaire LP, atrofische LP, annulaire atrofische LP, lichen planopilaris (omvattende Graham Little-Piccardi-Lassueursyndroom en frontaal fibroserende alopecie), lichen planus pigmentosus, en lichen planus pemphigoides (zie deze termen). Zeldzame mucosale LP omvat vulvovaginaal gingivaal syndroom en LP sialadenitis (zie deze termen)."},"pt":{"name":"Líquen plano raro","synonyms":["LP raro"],"definition":"O líquen plano (LP) é uma dermatose inflamatória comum caracterizada pelo desenvolvimento de placas ou pápulas violáceas pruríticas nas superfícies mucocutâneas. As erupções podem envolver a face, pescoço, membros, costas, genitais, língua, mucosa bucal, unhas e couro cabeludo. A LP compreende variantes raras que afetam a pele e a mucosa. A LP cutânea rara inclui LP linear (referente às distribuições blaschcóide e zosteriforme das lesões liquenóides), LP actínica, LP anular, LP atrófica, LP atrófica anular, líquen plano pilar (compreendendo a síndrome Graham Little-Piccardi-Lassueur e alopécia fibrosante frontal), líquen plano pigmentoso e líquen plano penfigóide (ver estes termos). A LP mucosa rara inclui a síndrome gengival vulvovaginal e a sialadenite LP (ver estes termos)."},"pl":{"name":"Rzadka postać liszaja płaskiego","synonyms":["Rzadka postać LP"]},"cs":{"name":"Vzácný lichen planus","synonyms":["Vzácný LP"]},"uk":{"name":"Рідкісний червоний плескатий лишай","synonyms":["Рідкісна LP"],"definition":"Lichen planus (LP) is a common inflammatory dermatosis characterized by the development of pruritic violaceous papules or plaques on mucocutaneous surfaces. Eruptions can involve the face, neck, limbs, back, genitalia, tongue, buccal mucosa, nails, and scalp. LP comprises rare variants affecting the skin and the mucosa. Rare cutaneous LP includes linear LP (referring to blaschkoid and zosteriform distributions of lichenoid lesions), actinic LP, annular LP, atrophic LP, annular atrophic LP, lichen planopilaris (comprising Graham Little-Piccardi-Lassueur syndrome and frontal fibrosing alopecia), lichen planus pigmentosus, and lichen planus pemphigoides (see these terms). Rare mucosal LP includes vulvovaginal gingival syndrome and LP sialadenitis (see these terms)."},"zh":{"name":"罕见性扁平苔藓","synonyms":["罕见性LP"],"definition":"扁平苔藓(LP)是一种常见的炎症性皮肤病，其特征是皮肤粘膜表面出现瘙痒性紫蓝色丘疹或斑块。皮疹可累及面部、颈部、四肢、背部、生殖器、舌头、颊粘膜、指甲和头皮。LP的罕见类别主要包括影响皮肤和黏膜这两种。罕见的皮肤扁平苔藓包括线形扁平苔藓(指苔藓样皮损的囊样和带状分布)、光化性扁平苔藓、环状扁平苔藓、萎缩性扁平苔藓、环状萎缩性扁平苔藓、毛发扁平苔藓(包括Graham Little-Piccardi-Lassueur综合征和额叶纤维性脱发)、色素性苔藓和类天疱疮苔藓(见该类词条)。罕见的黏膜扁平苔藓包括外阴阴道牙龈综合征和扁平苔藓涎腺炎(见该类词条)。"},"tr":{"name":"Nadir liken planus","synonyms":["Nadir LP"]}},"xrefs":[{"source":"MONDO","code":"16766","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679638","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12344","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79353,"name":"Epidermal disease","classifications":[187]}],"children":[{"orpha":254370,"name":"Rare cutaneous lichen planus","type":"Category"},{"orpha":254373,"name":"Rare mucosal lichen planus","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}