Treatment of Pemphigus Foliaceus
Rituximab combined with short-term corticosteroids is the most effective first-line treatment for pemphigus foliaceus, achieving 88% complete remission at 24 months compared to only 63% with corticosteroids alone. 1, 2
First-Line Treatment Options
Rituximab + Short-term Corticosteroids
- Initial rituximab dosing: 1000 mg IV on days 0 and 14
- Maintenance: 500 mg at month 12 and 18
- Combined with short-term prednisone:
- Moderate disease: 0.5 mg/kg/day tapered over 3 months
- Severe disease: 1 mg/kg/day tapered over 6 months
Corticosteroids with Adjuvant Immunosuppressants
If rituximab is unavailable or contraindicated:
Systemic Corticosteroids
- Prednisone 0.5-1 mg/kg/day
- Taper gradually based on clinical response
Adjuvant Immunosuppressants (added to reduce steroid requirements)
- Azathioprine: 1-3 mg/kg/day (check TPMT activity before starting)
- Mycophenolate mofetil: 1 g twice daily
- Allow at least 6 weeks before assessing efficacy of adjuvants
Mild Disease Management
For localized or mild disease:
- Topical corticosteroids: Clobetasol propionate 0.05% cream twice daily 3
- Can be effective as monotherapy in mild cases
- Complete healing of cutaneous lesions often occurs within 15 days
- Some patients may maintain remission with topical treatment alone
Second-Line and Refractory Disease Options
Intravenous Immunoglobulin (IVIG)
- Dosage: 2 g/kg divided over 3-5 days
- Frequency: Every 2-4 weeks
- Particularly useful in steroid-dependent patients 4
- Demonstrated steroid-sparing effect and ability to maintain prolonged clinical remission
Immunoadsorption
- Consider in recalcitrant cases where conventional therapy fails
- Effective in reducing circulating pathogenic antibody levels by up to 95% 5
- Best used in combination with treatments that suppress new antibody formation
Extracorporeal Photopheresis
- 2-day cycles every 2-4 weeks
- Can achieve complete remission in most patients 5, 1
- Allows for tapering of steroid doses
Treatment Monitoring and Adjustment
- Assess weekly to biweekly during induction phase, then monthly during maintenance
- Monitor:
- New blister formation
- Healing of existing lesions
- Complete blood count
- Liver and kidney function tests
- Blood glucose and lipid profile
Relapse Management
- For relapse after rituximab: administer additional 1000 mg IV rituximab
- Consider resuming or increasing glucocorticoid dose
- Subsequent rituximab infusions should not be given sooner than 16 weeks after previous infusion
Important Considerations
- Infection prophylaxis: Consider pneumocystis jirovecii pneumonia and herpes virus prophylaxis
- Bone health: Calcium and vitamin D supplementation; consider bisphosphonates if corticosteroid duration exceeds 3 months
- Pregnancy: Prednisolone is first-line; avoid mycophenolate mofetil, methotrexate, and cyclophosphamide
- Cyclosporine: Not recommended as an adjuvant drug based on evidence showing no advantage over corticosteroids alone 6
- Plasma exchange: Not recommended as routine treatment but may be considered in refractory cases when combined with corticosteroids and immunosuppressants 5
Treatment Outcomes
- Rituximab with short-term corticosteroids shows superior outcomes with 88% complete remission in pemphigus foliaceus patients at 24 months 2
- Significantly reduced cumulative steroid dose with rituximab (5800 mg vs 20,520 mg with prednisone alone) 7
- Lower incidence of grade 3/4 corticosteroid-related adverse events with rituximab plus prednisone (34%) compared to prednisone alone (67%) 7
The evidence strongly supports rituximab plus short-term corticosteroids as the optimal first-line treatment for pemphigus foliaceus, offering higher remission rates and reduced steroid-related complications.