{"title":"Therapeutic agents for Hailey-Hailey disease: A narrative review.","authors":"Kabir Sardana, Abhinav Bansal, Aishwarya Muddebihal, Ananta Khurana","doi":"10.25259/IJDVL_906_2024","DOIUrl":null,"url":null,"abstract":"<p><p>Hailey-Hailey disease (HHD) is an autosomal dominant genetic disorder of keratinocyte adhesion. It occurs due to mutations in ATP2C1, a gene on chromosome 3q21-24 which encodes human secretory pathway Ca2+/Mn2+ ATPase isoform 1, a calcium pump on the Golgi apparatus membrane. Recently, there has been a focus on certain pro-inflammatory cytokines such as IL-6 and IL-8 which play a role in HHD. Various triggers include excessive heat, menstruation, pregnancy, sweating, friction, exposure to sunlight and superficial infections. The therapy of the disorder is better understood by the varied pathogenetic steps and we aimed to comprehensively search for the various medical therapies used in HHD and align them with the existing knowledge on the pathogenesis and delineate them according to their major mode of action.</p>","PeriodicalId":50376,"journal":{"name":"Indian Journal of Dermatology Venereology & Leprology","volume":" ","pages":"1-8"},"PeriodicalIF":3.2000,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Indian Journal of Dermatology Venereology & Leprology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.25259/IJDVL_906_2024","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"DERMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Hailey-Hailey disease (HHD) is an autosomal dominant genetic disorder of keratinocyte adhesion. It occurs due to mutations in ATP2C1, a gene on chromosome 3q21-24 which encodes human secretory pathway Ca2+/Mn2+ ATPase isoform 1, a calcium pump on the Golgi apparatus membrane. Recently, there has been a focus on certain pro-inflammatory cytokines such as IL-6 and IL-8 which play a role in HHD. Various triggers include excessive heat, menstruation, pregnancy, sweating, friction, exposure to sunlight and superficial infections. The therapy of the disorder is better understood by the varied pathogenetic steps and we aimed to comprehensively search for the various medical therapies used in HHD and align them with the existing knowledge on the pathogenesis and delineate them according to their major mode of action.
期刊介绍:
The Indian Association of Dermatologists, Venereologists & Leprologists (IADVL) is the national association of Indian medical specialists who manage patients with skin disorders, sexually transmitted infections (STIs) or leprosy. The current member strength of the association is about 3800. The association works for the betterment of the specialty by holding academic meetings, printing a journal and publishing a textbook. The IADVL has several state branches, each with their own office bearers, which function independently within the constitution of the IADVL.
Established in 1940, the Indian Journal of Dermatology, Venereology and Leprology (IJDVL, ISSN 0378-6323) is the official publication of the IADVL (Indian Association of Dermatologists, Venereologists and Leprologists).