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Treatment Algorithm Suggestion for Hailey-Hailey Disease.

Nefeli Giannopoulou1, Olivia Mathot1, Bertrand Richert1

  • 1Dermatology Department, Université Libre de Bruxelles, University Hospital Brugmann and Saint-Pierre, Brussels, Belgium.

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Summary

Hailey-Hailey disease, a rare genetic skin disorder, presents challenges due to limited treatment options. This review systematically analyzes current and emerging therapies to guide clinical decisions for this chronic condition.

Keywords:
Hailey-Hailey diseasefamilial benign chronic pemphigusinterventional treatmentssystemic treatment

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Area of Science:

  • Dermatology
  • Genetics
  • Rare Diseases

Background:

  • Hailey-Hailey disease is a rare autosomal dominant genodermatosis affecting keratinocyte adhesion.
  • Caused by ATP2C1 gene mutations, it clinically manifests as blisters, erosions, and fissures in flexural areas.
  • The condition follows a chronic relapsing course with limited therapeutic options.

Purpose of the Study:

  • To systematically review established and emerging treatments for Hailey-Hailey disease.
  • To assess the evidence supporting various therapeutic options.
  • To propose a therapeutic algorithm for clinical decision-making.

Main Methods:

  • Systematic literature analysis of Hailey-Hailey disease treatments.
  • Evaluation of evidence strength for each treatment modality.
  • Focus on both pharmacological and procedural therapies.

Main Results:

  • A wide range of treatments exist, including topical, systemic, and procedural options.
  • Evidence for treatment effectiveness and safety is often limited due to a lack of large trials.
  • Non-pharmacological measures are crucial for managing the disease.

Conclusions:

  • Developing evidence-based guidelines for Hailey-Hailey disease is challenging.
  • A comprehensive understanding of treatment evidence is needed to guide clinical practice.
  • This review aims to provide a structured approach to managing Hailey-Hailey disease.