{"title":"Darier-White病伴感觉神经性听力损失一例报告","authors":"E. Henshaw, H. Ekpenyong, L. E. Ekafor","doi":"10.4314/rmj.v79i2.1","DOIUrl":null,"url":null,"abstract":"\n \n \nDarier-White disease (keratosis follicularis) is a rare autosomal dominant genodermatosis characterized by hyperkeratotic papules and plaques in seborrheic areas, often presenting with nail abnormalities and occasionally mucous membrane changes.It has been associated with neurocognitive/psychiatric disorders, ankylosing spondylitis, hidradenitis suppurativa and nephritis. Affected individuals also have an increased tendency to cutaneous infections. \nHowever, a thorough literature search showed no association with sensorineural hearing loss. We herein report the case of a 34-year-old Nigerian man with disfiguring Darier-White disease (DWD) associated with spondyloarthropathy and sensorineural hearing loss. We affirm that early diagnosis, prompt and appropriate therapy and adequate patient education can avert undesirable outcomes. \n \n \n","PeriodicalId":38181,"journal":{"name":"Rwanda Medical Journal","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2022-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Darier-White Disease with Sensorineural Hearing Loss – A Case Report\",\"authors\":\"E. Henshaw, H. Ekpenyong, L. E. Ekafor\",\"doi\":\"10.4314/rmj.v79i2.1\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"\\n \\n \\nDarier-White disease (keratosis follicularis) is a rare autosomal dominant genodermatosis characterized by hyperkeratotic papules and plaques in seborrheic areas, often presenting with nail abnormalities and occasionally mucous membrane changes.It has been associated with neurocognitive/psychiatric disorders, ankylosing spondylitis, hidradenitis suppurativa and nephritis. Affected individuals also have an increased tendency to cutaneous infections. \\nHowever, a thorough literature search showed no association with sensorineural hearing loss. We herein report the case of a 34-year-old Nigerian man with disfiguring Darier-White disease (DWD) associated with spondyloarthropathy and sensorineural hearing loss. We affirm that early diagnosis, prompt and appropriate therapy and adequate patient education can avert undesirable outcomes. \\n \\n \\n\",\"PeriodicalId\":38181,\"journal\":{\"name\":\"Rwanda Medical Journal\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-06-30\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Rwanda Medical Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4314/rmj.v79i2.1\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rwanda Medical Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4314/rmj.v79i2.1","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
Darier-White Disease with Sensorineural Hearing Loss – A Case Report
Darier-White disease (keratosis follicularis) is a rare autosomal dominant genodermatosis characterized by hyperkeratotic papules and plaques in seborrheic areas, often presenting with nail abnormalities and occasionally mucous membrane changes.It has been associated with neurocognitive/psychiatric disorders, ankylosing spondylitis, hidradenitis suppurativa and nephritis. Affected individuals also have an increased tendency to cutaneous infections.
However, a thorough literature search showed no association with sensorineural hearing loss. We herein report the case of a 34-year-old Nigerian man with disfiguring Darier-White disease (DWD) associated with spondyloarthropathy and sensorineural hearing loss. We affirm that early diagnosis, prompt and appropriate therapy and adequate patient education can avert undesirable outcomes.
期刊介绍:
The Rwanda Medical Journal (RMJ), is a Not-For-Profit scientific, medical, journal that is published entirely online in open-access electronic format. The RMJ is an interdisciplinary research journal for publication of original work in all the major health disciplines. Through a rigorous process of evaluation and peer review, The RMJ strives to publish original works of high quality for a diverse audience of healthcare professionals. The Journal seeks to deepen knowledge and advance scientific discovery to improve the quality of care of patients in Rwanda and internationally.