From the Guidelines
The management of Hailey-Hailey disease should prioritize topical photodynamic therapy (PDT) with 5-aminolaevulinic acid (ALA) and red light, as it has shown histological clearance and no recurrences at 19-25 months in a case series 1. The treatment of Hailey-Hailey disease involves a combination of preventive measures and therapies to control flares.
- Gentle skin care with mild cleansers
- Avoiding irritants
- Keeping affected areas dry and cool to prevent sweating Topical treatments are the mainstay of therapy, including:
- Potent corticosteroids
- Calcineurin inhibitors
- Antimicrobials for secondary infections However, topical PDT with ALA and red light has emerged as a promising treatment option, with a case series demonstrating histological clearance and no recurrences at 19-25 months 1. For more severe cases, oral antibiotics or systemic retinoids may be needed, while surgical options like dermabrasion, laser therapy, or excision with skin grafting may be considered for persistent localized disease. The use of topical PDT with ALA and red light is supported by the most recent and highest quality study, making it the recommended first-line treatment for Hailey-Hailey disease 1.
From the Research
Management of Hailey-Hailey Disease
The management of Hailey-Hailey disease is challenging and includes various treatment options, such as:
- Topical medications, including corticosteroids, calcineurin inhibitors, and antibiotics 2
- Systemic medications, including antibiotics, corticosteroids, immunosuppressants, retinoids, and immunobiologicals 2
- Procedures, such as botulinum toxin, laser, and surgery 2
- Oral retinoid therapy, such as acitretin, which has been reported to improve disease manifestations in refractory cases 3
- Topical tacrolimus ointment and clobetasol propionate foam, which have been found to be effective in controlling Hailey-Hailey outbreaks 4
- Biologics and small-molecule inhibitors, such as dupilumab, apremilast, and upadacitinib, which may offer a treatment alternative for patients with refractory Hailey-Hailey disease 5
Treatment Goals
The goals of treatment for Hailey-Hailey disease include:
- Controlling heat, sweat, and friction 2
- Managing symptoms, such as pruritus, burning, and pain 2
- Preventing secondary infections 2
- Improving quality of life and reducing the psychological and social impact of the disease 6
Treatment Challenges
The treatment of Hailey-Hailey disease is challenging due to: