海利-海利病的治疗药物：叙述性综述。

IF 3.2 4区医学 Q2 DERMATOLOGY

Indian Journal of Dermatology Venereology & Leprology Pub Date : 2024-09-24 DOI:10.25259/IJDVL_906_2024

Kabir Sardana, Abhinav Bansal, Aishwarya Muddebihal, Ananta Khurana

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引用次数: 0

摘要

海利-海利病（Hailey-Hailey disease，HHD）是一种常染色体显性遗传的角质细胞粘附性疾病。该基因编码人类分泌途径 Ca2+/Mn2+ ATPase 异构体 1，是高尔基体膜上的一种钙泵。最近，人们开始关注某些促炎细胞因子，如 IL-6 和 IL-8，它们在 HHD 中发挥着作用。各种诱发因素包括过热、月经、怀孕、出汗、摩擦、日光照射和急性感染。我们的目标是全面搜索用于 HHD 的各种医学疗法，并将其与现有的发病机制知识进行比对，同时根据其主要作用模式对其进行划分。

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Therapeutic agents for Hailey-Hailey disease: A narrative review.

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.

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来源期刊

Indian Journal of Dermatology Venereology & Leprology 医学-皮肤病学

CiteScore

2.10

自引率

10.30%

发文量

247

审稿时长

6-12 weeks

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).