Treatment of Hailey-Hailey Disease
The most effective treatment approach for Hailey-Hailey disease involves topical steroids and antimicrobials as first-line therapy, with refractory cases benefiting from oral antibiotics, excisional procedures, and botulinum toxin A. 1
Disease Overview
- Hailey-Hailey disease (familial benign chronic pemphigus) is a rare autosomal dominant blistering dermatosis with an incidence of approximately 1/50,000, characterized by flaccid vesicles and blisters on erythematous skin in intertriginous regions 2
- The disease typically presents between the third and fourth decades of life with a chronic relapsing course that can significantly impact quality of life 2
- Common affected areas include retroauricular folds, lateral neck, axillae, umbilicus, inguinal, and perianal regions 2
First-Line Treatment Options
Topical Therapies
- Topical corticosteroids (mild to moderate potency) are the mainstay of initial treatment to reduce inflammation and provide symptom relief 1
- Topical antimicrobials are essential to prevent and treat secondary infections, which frequently complicate the disease 1
- Patients should be advised to avoid friction and sweating by wearing light cotton clothes and using soothing compresses followed by topical corticosteroid application 3
Second-Line Treatment Options
Systemic Medications
- Oral antibiotics are recommended for more extensive disease or when secondary infections are present 1
- Systemic corticosteroids may be necessary for severe flares but should be used cautiously due to side effects with long-term use 1
Procedural Interventions
- Laser ablation has shown significant benefit for refractory disease and can provide longer periods of remission 4
- Botulinum toxin A injections are effective, particularly in intertriginous areas, by reducing sweating which can trigger flares 1
- Photodynamic therapy has demonstrated variable success in managing resistant cases 4
Treatment for Severe Refractory Disease
Advanced Systemic Options
- Immunosuppressive agents such as dapsone, cyclosporine, and azathioprine have been used with varying degrees of success for moderate to severe disease 1
- Hydroxyurea has shown promising results in a case report, with complete and durable response over a 4-year period 3
- Biologics and small-molecule inhibitors (including dupilumab, apremilast, upadacitinib, abrocitinib, adalimumab, and etanercept) may offer treatment alternatives for refractory cases, though larger clinical trials are needed 5
Surgical Interventions
- Dermabrasion can be effective for localized recalcitrant disease 4
- Excisional procedures with skin grafting represent a last resort option for resistant localized disease 3
Treatment Pitfalls and Considerations
- No single treatment regimen works for all patients, requiring an individualized approach 1
- Secondary infections (bacterial, viral, or fungal) are common complications and may require specific antimicrobial therapy 3
- Sun exposure can exacerbate the condition; patients should be advised to avoid sun and use SPF 50 sunscreen 3
- Treatment response is often temporary, with disease following a relapsing-remitting pattern requiring ongoing management 2