Diagnostic and Therapeutic Approaches to Vulvar Hailey-Hailey Disease

Abstract

Introduction

Vulvar Hailey-Hailey disease (HHD) is a rare genodermatosis characterized by chronic inflammation, infection, and blistering which may affect the vulva. Vulvar HHD and other vulvar dermatoses display broad histomorphologic overlap, often posing a challenge to clinicians while diagnosing and crafting treatment plans. Moreover, there is no comprehensive literature synthesis on vulvar HHD to clarify the current synthesis. The aim of this review is to identify, categorize, and summarize diagnostic methodologies, clinical features, therapeutic interventions, and reported outcomes for vulvar HHD. The protocol has been developed in accordance with the PRISMA-ScR guidelines.

Method and Analysis

An electronic search will be conducted across various databases, including PubMed/MEDLINE, Embase, and Scopus. Grey literature, conference abstracts, and reference lists of included studies will also be reviewed. Two independent reviews will identify studies for inclusion, and discrepancies will be resolved through consensus or a third reviewer. Two researchers will extract data from each paper. Data will be collected about (1) general study characteristics, (2) diagnostic methods, (3) treatment modalities, and (4) reported clinical characteristics and outcomes. Descriptive statistics and a narrative synthesis will be presented.

Ethics and Dissemination

The findings of this scoping review will be presented at relevant academic conferences and will be published in a peer-reviewed dermatology, women’s health, or gynecology-focused journal. Moreover, this review aims to inform clinicians and future reviewers about novel diagnostic methodologies,  therapeutic interventions, and reported outcomes for Vulvar Hailey-Hailey Disease. This study does not involve human subjects and therefore does not require IRB approval.