Treatment Protocol for Pemphigus Vulgaris

First-Line Therapy

The British Journal of Dermatology recommends starting with oral prednisolone at 1 mg/kg/day in most cases; use 0.5-1 mg/kg/day for milder disease 1
If no response within 5-7 days, increase dose in 50-100% increments until disease control is achieved 2
For severe disease or if doses above 1 mg/kg/day are required, consider pulsed intravenous corticosteroids (methylprednisolone 250-1000 mg or equivalent) 2, 4
Once remission is achieved (no new lesions and healing of existing ones), taper dose gradually with the aim to reduce to 10 mg daily or less 1
Assess risk of osteoporosis immediately and implement preventive measures 1
Azathioprine: 2-3 mg/kg/day (if TPMT normal) 1, 5
Mycophenolate mofetil: 2-3 g/day in divided doses 1, 6

Consider switching to an alternate corticosteroid-sparing agent if treatment failure with first-line adjuvant drug 1
For patients with gastrointestinal symptoms from mycophenolate mofetil, switch to mycophenolic acid 720-1080 mg twice daily 6
Treatment failure is defined as continued disease activity or failure to heal despite 3 weeks of prednisolone 1.5 mg/kg/day, or 12 weeks of azathioprine (2.5 mg/kg/day), mycophenolate mofetil (1.5 g twice daily), cyclophosphamide (2 mg/kg/day), or methotrexate (20 mg/week) 1, 4

For refractory cases, consider cyclophosphamide (oral or intravenous pulse therapy) 7
Immunoadsorption 1
Intravenous immunoglobulin 1
Methotrexate 1
Plasmapheresis or plasma exchange 1

Disease control is defined as no new lesions and the onset of healing in pre-existing ones 2
Monitor for clinical improvement (usually seen within days) and complete healing (typically 3-8 weeks) 3
Immunofluorescence titers fall with treatment but lag behind clinical improvement 3

Avoid premature treatment withdrawal; relapse rates are high initially with 47% of successfully treated patients relapsing when treatment is stopped after 1 year 3
Be aware of the latent period (6-8 weeks) before effects of azathioprine and mycophenolate mofetil are seen 6, 8
Corticosteroid side effects are common and dose-related, with one study estimating that up to 77% of deaths were corticosteroid-related 1
For pregnant patients, prednisolone is preferred as it is 90% inactivated by the placenta, unlike betamethasone and dexamethasone 9
Infection and sepsis are significant risks and major causes of mortality; maintain vigilance for signs of infection 9