{"orpha":90282,"name":"Hypertrophic or verrucous lupus erythematosus","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Hypertrophic or verrucous lupus erythematosus is a rare type of chronic cutaneous lupus erythematosus characterized by the appearance of lesions on sun-exposed areas (frequently the extensor surfaces of forearms, face, upper trunk) which vary from squamous violet, painful papules and blackish hyperkeratotic ulcers to depigmented atrophic plaques on the back, hyperkeratotic papules on upper extremities, and disseminated keratoacanthoma-like papulonodular verrucous lesions. Classic discoid lesions and squamous cell carcinoma may be associated. Histopathology reveals follicular plugging, liquefactive basal layer degeneration and a perivascular lymphocytic infiltrate.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90282/","orphanet_url":"https://www.orpha.net/en/disease/detail/90282","names":{"fr":{"name":"Lupus érythémateux verruqueux ou hypertrophique","definition":"Type rare de lupus érythémateux cutané chronique caractérisé par l'apparition de lésions sur les zones exposées au soleil (fréquemment les surfaces d'extension des avant-bras, du visage, de la partie supérieure du tronc) qui vont de papules squameuses violacées et douloureuses et d'ulcères hyperkératosiques noirâtres à des plaques atrophiques dépigmentées dans le dos, des papules hyperkératosiques sur les extrémités supérieures et des lésions verruqueuses papulonodulaires disséminées ressemblant à un kératoacanthome. Des lésions discoïdes classiques et un carcinome épidermoïde peuvent être associés. L'histopathologie révèle un bouchon folliculaire, une dégénérescence liquéfactive de la couche basale et un infiltrat lymphocytaire périvasculaire."},"de":{"name":"Lupus erythematosus, hypertropher oder verruköser"},"es":{"name":"Lupus eritematoso hipertrófico o verrugoso","definition":"Es un tipo de lupus eritematoso cutáneo crónico, poco frecuente, caracterizado por la aparición de lesiones cutáneas en áreas expuestas al sol (con frecuencia las superficies extensoras de los antebrazos, la cara y el tronco superior) que varían desde unas pápulas escamosas dolorosas de color violáceo y unas úlceras hiperqueratósicas oscuras hasta placas atróficas despigmentadas en la espalda, unas pápulas hiperqueratósicas en las extremidades superiores y unas lesiones verrugosas papulonodulares diseminadas tipo queratoacantoma. También pueden estar asociadas lesiones clásicas discoides y carcinoma de células escamosas. La histopatología revela taponamiento folicular, degeneración licuefactiva de la capa basal y un infiltrado linfocítico perivascular."},"it":{"name":"Lupus eritematoso ipertrofico o verrucoso","definition":"Il lupus eritematoso ipertrofico o verrucoso è una forma rara di lupus eritematoso cutaneo cronico, caratterizzata dalla comparsa di lesioni nelle zone esposte al sole (spesso le superfici estensorie degli avambracci, il viso e la parte superiore del tronco), che comprendono papule viola desquamative dolorose, ulcere ipercheratosiche nerastre, placche atrofiche depigmentate sulla schiena, papule ipercheratosiche sugli arti superiori e lesioni verrucose papulo-nodulari diffuse simili ai cheratoacantomi. Può associarsi a lesioni discoidi classiche e al carcinoma a cellule squamose. Gli esami istopatologici evidenziano occlusione follicolare, degenerazione con liquefazione dello strato basale ed infiltrato linfocitico perivascolare."},"nl":{"name":"Hypertrofische of verruceuze lupus erythematosus","definition":"Hypertrofische of verruceuze lupus erythematosus is een zeldzaam type van chronische cutane lupus erythematosus, en wordt gekarakteriseerd door laesies die verschijnen op lichaamsdelen die worden blootgesteld aan de zon (vaak de strekzijde van de voorarmen, het gelaat, het bovenste deel van de romp), en die variëren van squameuze, paarse, pijnlijke papels en zwarte hyperkeratotische zweren tot gedepigmenteerde atrofische plaques op de rug, hyperkeratotische papels op de bovenste ledematen, en verspreide keratoacanthoom-achtige papulonodulaire verruceuze letsels. Klassieke discoïde laesies en plaveiselcelcarcinoom kunnen geassocieerd zijn. Histopathologie toont obstructie van follikels, degeneratie van de basale membraan door liquefactie, en een perivasculair lymfocytair infiltraat."},"pt":{"name":"Lúpus eritematoso hipertrófico ou verrucoso"},"pl":{"name":"Odmiana przerosła ogniskowego tocznia rumieniowatego"},"cs":{"name":"Hypertrofický nebo verukózní lupus erythematodes"},"uk":{"name":"Гіпертрофічний або верукозний червоний вовчак","definition":"Hypertrophic or verrucous lupus erythematosus is a rare type of chronic cutaneous lupus erythematosus characterized by the appearance of lesions on sun-exposed areas (frequently the extensor surfaces of forearms, face, upper trunk) which vary from squamous violet, painful papules and blackish hyperkeratotic ulcers to depigmented atrophic plaques on the back, hyperkeratotic papules on upper extremities, and disseminated keratoacanthoma-like papulonodular verrucous lesions. Classic discoid lesions and squamous cell carcinoma may be associated. Histopathology reveals follicular plugging, liquefactive basal layer degeneration and a perivascular lymphocytic infiltrate."},"zh":{"name":"肥厚性或疣状红斑狼疮"},"tr":{"name":"Hipertrofik veya verrüköz lupus eritematozus"}},"xrefs":[{"source":"ICD-10","code":"L93.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB51.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1537801570"},{"source":"MONDO","code":"0019559","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1274838","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":163531,"name":"Chronic cutaneous lupus erythematosus","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}