From the Guidelines
Treatment for a flare-up of Hailey-Hailey disease should prioritize reducing inflammation and preventing secondary infections, with topical corticosteroids being a first-line approach. Although the provided evidence focuses on pemphigus vulgaris, the principles of managing autoimmune blistering diseases can be applied to Hailey-Hailey disease, which is also characterized by skin blistering. For mild flares, topical corticosteroids such as betamethasone or clobetasol applied twice daily for 1-2 weeks can reduce inflammation, similar to the approach suggested for pemphigus vulgaris, where oral prednisolone is started at 1 mg kg-1 per day 1.
Key Considerations
- Topical antibiotics like clindamycin or mupirocin may be added if there's evidence of bacterial infection, highlighting the importance of assessing for secondary infections in the management of Hailey-Hailey disease.
- For more severe flares, systemic treatments may be necessary, and while the evidence provided does not directly address Hailey-Hailey disease, the use of corticosteroids and adjuvant immunosuppressants in pemphigus vulgaris suggests that similar approaches could be considered in severe cases of Hailey-Hailey disease, under the guidance of a dermatologist.
- Keeping the affected areas clean and dry is crucial, as moisture and friction worsen symptoms, which aligns with general principles of wound care and management of skin conditions.
Treatment Options
- Topical corticosteroids for mild flares
- Topical antibiotics for suspected bacterial infections
- Systemic antibiotics for more severe infections
- Antifungal medications if fungal overgrowth is present
- Oral retinoids, botulinum toxin injections, or laser therapy for persistent cases, as these can address underlying factors contributing to the disease.
Given the nature of Hailey-Hailey disease and the importance of managing flares effectively to improve quality of life and reduce morbidity, a tailored approach considering the severity of the flare and the patient's overall condition is essential, and should be guided by the most recent and highest quality evidence available, even if that evidence is from related conditions like pemphigus vulgaris 1.
From the Research
Treatment Options for Hailey-Hailey Disease
The treatment for Hailey-Hailey disease is challenging and often requires a combination of therapies. Some of the treatment options include:
- Topical medications such as corticosteroids, calcineurin inhibitors, and antibiotics 2, 3, 4
- Systemic medications such as antibiotics, corticosteroids, immunosuppressants, retinoids, and immunobiologicals 3, 4, 5
- Procedures such as botulinum toxin, laser, and surgery 3, 4, 6
- Intermittent therapy with clobetasol propionate 0.05% foam for patients who break through suppressive therapy with tacrolimus 2
- Alternating tacrolimus with clobetasol propionate 0.05% foam every 6 weeks for patients with frequent outbreaks 2
- Apremilast, which has shown efficacy in some cases of Hailey-Hailey disease 5
Specific Treatment Regimens
Some specific treatment regimens that have been reported to be effective include:
- Applying topical tacrolimus 0.1% ointment twice a day to affected areas 2
- Using clobetasol propionate 0.05% foam intermittently for patients who break through suppressive therapy with tacrolimus 2
- Alternating tacrolimus with clobetasol propionate 0.05% foam every 6 weeks for patients with frequent outbreaks 2
- Administering apremilast orally, which has shown efficacy in some cases of Hailey-Hailey disease 5
Challenges in Treatment
The treatment of Hailey-Hailey disease is challenging due to the lack of controlled clinical trials to support the choice of the best treatment 4. Additionally, the disease can be recalcitrant to conventional therapy, and multiple treatment modalities may be needed to obtain benefit 3.