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.