大范围生殖器外奇拉格氏病:罕见病例报告

Rajesh Rajagopalan
{"title":"大范围生殖器外奇拉格氏病:罕见病例报告","authors":"Rajesh Rajagopalan","doi":"10.18203/issn.2455-4529.intjresdermatol20240972","DOIUrl":null,"url":null,"abstract":"Lichen sclerosus et atrophicus (LSA), also called as Csillag’s disease, is characterized by small, shiny, porcelain white, sclerotic papules, plaques and atrophic patches that can occur at any site on the skin including the mucosa. Most commonly found in genitalia, rarely occurring on extragenital skin. Predominantly seen in women with bimodal age distribution. Predominantly an interface dermatoses histopathologically consisting of epidermal atrophy, sclerosis /homogenization of collagen fibers and lichenoid inflammatory infiltrates in dermis. No effective treatment till date although topical steroids, calcineurin inhibitors, topical retinoids and systemic agents like hydroxychloroquine, mexthotrexate, PUVA shows varying inconsistent results. Here we report a case of 36-year-old female having LSA with extensive extra vulval involvement including face. No any other co-existing skin disorders. She showed partial improvement use of physical sunscreens, systemic antimalarials, oral methotrexate and antioxidants.","PeriodicalId":14331,"journal":{"name":"International Journal of Research in Dermatology","volume":"41 22","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Extensive extra genital Csillag’s disease: a rare case report\",\"authors\":\"Rajesh Rajagopalan\",\"doi\":\"10.18203/issn.2455-4529.intjresdermatol20240972\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Lichen sclerosus et atrophicus (LSA), also called as Csillag’s disease, is characterized by small, shiny, porcelain white, sclerotic papules, plaques and atrophic patches that can occur at any site on the skin including the mucosa. Most commonly found in genitalia, rarely occurring on extragenital skin. Predominantly seen in women with bimodal age distribution. Predominantly an interface dermatoses histopathologically consisting of epidermal atrophy, sclerosis /homogenization of collagen fibers and lichenoid inflammatory infiltrates in dermis. No effective treatment till date although topical steroids, calcineurin inhibitors, topical retinoids and systemic agents like hydroxychloroquine, mexthotrexate, PUVA shows varying inconsistent results. Here we report a case of 36-year-old female having LSA with extensive extra vulval involvement including face. No any other co-existing skin disorders. She showed partial improvement use of physical sunscreens, systemic antimalarials, oral methotrexate and antioxidants.\",\"PeriodicalId\":14331,\"journal\":{\"name\":\"International Journal of Research in Dermatology\",\"volume\":\"41 22\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-04-24\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International Journal of Research in Dermatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.18203/issn.2455-4529.intjresdermatol20240972\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Research in Dermatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.18203/issn.2455-4529.intjresdermatol20240972","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

硬皮病和萎缩性苔藓(LSA)又称克西拉格氏病,其特征是皮肤上包括粘膜在内的任何部位都可能出现小的、发亮的、瓷白色的硬化性丘疹、斑块和萎缩性斑块。最常见于生殖器,很少发生在生殖器外皮肤。主要见于女性,年龄呈双峰分布。组织病理学上主要是一种界面皮肤病,包括表皮萎缩、胶原纤维硬化/同质化和真皮层苔藓样炎性浸润。虽然外用类固醇激素、钙调磷酸酶抑制剂、外用维甲酸和全身用药如羟氯喹、甲氨喋呤、PUVA 等效果不一,但至今仍无有效治疗方法。在此,我们报告了一例 36 岁女性 LSA 患者,其外阴广泛受累,包括面部。患者无其他并存皮肤疾病。使用物理防晒霜、全身用抗疟药、口服甲氨蝶呤和抗氧化剂后,她的病情得到部分改善。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Extensive extra genital Csillag’s disease: a rare case report
Lichen sclerosus et atrophicus (LSA), also called as Csillag’s disease, is characterized by small, shiny, porcelain white, sclerotic papules, plaques and atrophic patches that can occur at any site on the skin including the mucosa. Most commonly found in genitalia, rarely occurring on extragenital skin. Predominantly seen in women with bimodal age distribution. Predominantly an interface dermatoses histopathologically consisting of epidermal atrophy, sclerosis /homogenization of collagen fibers and lichenoid inflammatory infiltrates in dermis. No effective treatment till date although topical steroids, calcineurin inhibitors, topical retinoids and systemic agents like hydroxychloroquine, mexthotrexate, PUVA shows varying inconsistent results. Here we report a case of 36-year-old female having LSA with extensive extra vulval involvement including face. No any other co-existing skin disorders. She showed partial improvement use of physical sunscreens, systemic antimalarials, oral methotrexate and antioxidants.
求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信