Progress in the treatment of Hailey-Hailey disease

Abstract

Familial benign chronic pemphigus, also known as Hailey-Hailey disease, is an autosomal dominant disease having genetic features that follow Mendelian inheritance principles. HHD is a rare disease that affects approximately one out of every 50,000 people. Clinically, it presents as blisters, fissures, erosions, and vegetations with significant pruritus or pain in skin folds or reversal areas of the skin. In the presence of heat, localized sweating, or exercise, patients with HHD may suffer repeated or worsened symptoms. Furthermore, concurrent bacterial, fungal, and viral infections may aggravate localized lesions. HHD may raise the likelihood of problems such as insomnia, anxiety, and depression. There are no treatment guidelines or large clinical trials for HHD. This could be attributed to the low prevalence of HHD. Although the pathogenesis of HHD is well understood, there are no specific or curative treatment options available. The majority of current treatment focuses on symptomatic relief. In this article, we will discuss the most recent advancements in HHD research.