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Hailey-Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms
Mahesh Mathur1, Sumit Paudel1, Nabita Bhattarai1
1Department of Dermatology College of Medical Sciences Teaching Hospital Bharatpur Nepal.
Health Science Reports
|July 15, 2025
Summary
Hailey-Hailey disease (HHD) is a rare genetic skin disorder causing painful blisters. Current management focuses on symptom control and reducing recurrence, as no cure exists.
Area of Science:
- Dermatology
- Genetics
- Molecular Biology
Background:
- Hailey-Hailey disease (HHD), also known as familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis.
- It presents as chronic, painful, erosive plaques and fissures in friction-prone areas.
- Pathogenesis involves ATP2C1 gene mutations affecting Golgi apparatus calcium and manganese regulation.
Purpose of the Study:
- To review the efficacy of current treatments for Hailey-Hailey disease.
- To explore the therapeutic mechanisms underlying HHD management.
Main Methods:
- Comprehensive literature review of HHD treatments.
- Analysis of clinical and histopathological features.
- Focus on therapeutic mechanisms and management guidance.
Main Results:
- Diagnosis relies on clinical presentation and characteristic histopathology (e.g., "dilapidated brick wall").
- No definitive cure exists; management aims to control symptoms and minimize recurrence.
- Various topical, systemic, laser, and surgical therapies have been reported.
Conclusions:
- HHD management requires a multi-faceted approach.
- Understanding therapeutic mechanisms is crucial for effective treatment.
- This review provides guidance for dermatologists managing HHD.
