{"orpha":330064,"name":"Chronic actinic dermatitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Actinic reticuloid","Chronic photosensitivity dermatitis"],"definition":"Chronic actinic dermatitis (CAD) is an immunologically mediated photodermatosis usually observed in temperate climates and that typically develops in middle-aged to elderly males. CAD is characterized by eczematous and often lichenified pruritic patches and confluent plaques located predominantly on sun-exposed areas with notable sparing of eyelids, skin folds, and postauricular skin. It is often accompanied by multiple contact allergies and usually occurs in a background of either atopic, contact allergic, or seborrheic dermatitis, although it can occur <i>de novo</i>. Resolution of photosensitivity is reported in up to 50% of individuals after 15 years or more, with contact allergies persisting.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-330064/","orphanet_url":"https://www.orpha.net/en/disease/detail/330064","names":{"fr":{"name":"Dermatite actinique chronique","synonyms":["Actinoréticulose"],"definition":"Photodermatose à médiation immunitaire généralement observée dans les climats tempérés affectant typiquement les hommes d'âge moyen à avancé. La dermatite actinique chronique (DAC) se caractérise par des plaques prurigineuses eczémateuses et souvent lichénifiées et des plaques confluentes situées principalement sur les zones exposées au soleil, épargnant les paupières, les plis cutanés et la zone rétro-auriculaire. Elle s'accompagne souvent de nombreuses allergies de contact et survient généralement dans un contexte de dermatite atopique, allergique de contact ou séborrhéique, bien qu'elle puisse survenir <i>de novo</i>. La photosensibilité se résorbe chez 50&nbsp;% des individus après 15&nbsp;ans ou plus, alors que les allergies de contact persistent."},"de":{"name":"Dermatitis, aktinische chronische","synonyms":["Chronische photosensitive Dermatitis","Retikuloid, aktinisches"]},"es":{"name":"Dermatitis actínica crónica","synonyms":["Dermatitis fotosensible crónica","Reticuloide actínico"],"definition":"Es una fotodermatosis inmunomediada que suele observarse en climas templados y que se desarrolla típicamente en varones de edad media o avanzada. Se caracteriza por máculas de aspecto eccematoso, con frecuencia pruriginosas y liquenificadas, y placas confluentes localizadas preferentemente en las áreas fotoexpuestas, respetando los párpados, los pliegues cutáneos y la región retroauricular. Se asocia con frecuencia a múltiples alergias de contacto y suele presentarse, dentro de un contexto de dermatitis atópica, alérgica de contacto o seborreica, aunque también puede desarrollarse <i>de novo</i>. En hasta un 50% de los casos, transcurridos unos 15 años o más, se ha descrito una remisión de la fotosensibilidad, aunque suelen persistir las alergias de contacto."},"it":{"name":"Dermatite actinica cronica","synonyms":["Dermatite cronica da fotosensibilità","Reticuloide attinico"],"definition":"La dermatite attinica cronica (CAD) è una fotodermatosi immuno-mediata, che si osserva di solito nei climi temperati; in genere colpisce i maschi di mezza età o quelli anziani. È caratterizzata da aree di cute eczematosa e, spesso, da chiazze lichenificate e pruriginose e placche convergenti localizzate per lo più nelle aree della cute esposta al sole; le palpebre, le pieghe cutanee e la cute postauricolare sono normali. Spesso si associa ad allergie da contatto e ad una storia di dermatite atopica, o seborroica, o da allergia da contatto; può insorgere anche de novo. Nel 50% dei soggetti, la fotosensibilità scompare dopo 15 anni o poco più, mentre persistono le allergie da contatto."},"nl":{"name":"Chronische actinische dermatitis","synonyms":["Actinisch reticuloïd","Chonische lichtgevoeligheid-dermatitis"],"definition":"Chronische actinische dermatitis (CAD) is een immunologisch gemedieerde fotodermatose die meestal wordt waargenomen in gematigde klimaten en die doorgaans ontwikkelt in middelbare tot oude mannen. CAD wordt gekarakteriseerd door eczemateuze en vaak gelichenificeerde pruritische plekken en confluente plaques die zich voornamelijk bevinden op plaatsen die worden blootgesteld aan de zon, terwijl de oogleden, huidplooien en postauriculaire huid opvallend gespaard blijven. De aandoening wordt vaak vergezeld door meerdere contactallergieën en komt meestal voor in een achtergrond van atopische, contactallergische of seborrhoïsche dermatitis, hoewel ze ook <i>de novo</i> kan voorkomen. Bij nagenoeg 50%van de patiënten wordt remissie van fotosensitiviteit na 15 jaar of meer gerapporteerd, terwijl contactallergieën aanhouden."},"pt":{"name":"Dermatite actínica crónica","synonyms":["Dermatite fotossensitiva crónica","Reticuloide actínico"]},"pl":{"name":"Przewlekłe posłoneczne zapalenie skóry","synonyms":["Actinic reticuloid","Przewlekłe zapalenie skóry z fotonadwrażliwości"]},"cs":{"name":"Chronická aktinická dermatitida","synonyms":["Aktinický retikuloid","Fotosenzitivní dermatitida"]},"uk":{"name":"Хронічний актинічний дерматит","synonyms":["Актинічний ретикулоїд","Хронічний фоточутливий дерматит"],"definition":"Chronic actinic dermatitis (CAD) is an immunologically mediated photodermatosis usually observed in temperate climates and that typically develops in middle-aged to elderly males. CAD is characterized by eczematous and often lichenified pruritic patches and confluent plaques located predominantly on sun-exposed areas with notable sparing of eyelids, skin folds, and postauricular skin. It is often accompanied by multiple contact allergies and usually occurs in a background of either atopic, contact allergic, or seborrheic dermatitis, although it can occur <i>de novo</i>. Resolution of photosensitivity is reported in up to 50% of individuals after 15 years or more, with contact allergies persisting."},"zh":{"name":"慢性光化性皮炎","synonyms":["慢性光敏性皮炎"],"definition":"慢性光化性皮炎（CAD）是一种免疫介导的光敏性皮肤病，通常在温带气候地区观察到，并且通常发生在中老年男性。CAD的特征是湿疹，通常是苔藓样瘙痒斑和融合斑块，主要位于阳光照射区域，明显保留眼睑、皮肤皱褶和耳后皮肤。它通常伴有多发性接触性过敏，通常发生在有特应性、接触性过敏或脂溢性皮炎的情况下，尽管它可能发生从头变异。据报道，在15年或更长时间后，高达50%的人会出现光敏反应，并持续存在接触性过敏。"},"tr":{"name":"Kronik aktinik dermatit","synonyms":["Aktinik retiküloit","Kronik fotosensitivite dermatiti"]}},"xrefs":[{"source":"ICD-10","code":"L57.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EJ30.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"248339081"},{"source":"MONDO","code":"0018025","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0282309","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79390,"name":"Rare photodermatosis","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":16.7,"area":"United Kingdom","status":"Validated","source":"15813653[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"1-5 / 10 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-10-22","source":"11843205[PMID]_24891057[PMID]_29530872[PMID]_7944510[PMID]","terms":[{"hpo":"HP:0000992","label":"Cutaneous photosensitivity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000964","label":"Eczematoid dermatitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025092","label":"Epidermal acanthosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030350","label":"Erythematous papule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025127","label":"Actinic keratosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001053","label":"Hypopigmented skin patches","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007573","label":"Late onset atopic dermatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100725","label":"Lichenification","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007505","label":"Progressive hyperpigmentation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003193","label":"Allergic rhinitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001019","label":"Erythroderma","frequency":"Very rare (<4-1%)","criteria":null}]}}